Published Application/Species/Sample/Dilution | Reference |
---|
- immunohistochemistry knockout validation; mouse; 1:1000; loading ...; fig 8d
| Araki K, Araki A, Honda D, Izumoto T, Hashizume A, Hijikata Y, et al. TDP-43 regulates early-phase insulin secretion via CaV1.2-mediated exocytosis in islets. J Clin Invest. 2019;130:3578-3593 pubmed publisher
|
- immunohistochemistry knockout validation; mouse; 1:500; loading ...; fig 1b
| Laclair K, Donde A, Ling J, Jeong Y, Chhabra R, Martin L, et al. Depletion of TDP-43 decreases fibril and plaque β-amyloid and exacerbates neurodegeneration in an Alzheimer's mouse model. Acta Neuropathol. 2016;132:859-873 pubmed
|
- immunohistochemistry - paraffin section; human; 1:1000; loading ...; fig 3e
| Lloyd G, Trejo Lopez J, Xia Y, McFarland K, Lincoln S, Ertekin Taner N, et al. Prominent amyloid plaque pathology and cerebral amyloid angiopathy in APP V717I (London) carrier - phenotypic variability in autosomal dominant Alzheimer's disease. Acta Neuropathol Commun. 2020;8:31 pubmed publisher
|
- RNA immunoprecipitation; rat; loading ...; fig 5h
| Keihani S, Kluever V, Mandad S, Bansal V, Rahman R, Fritsch E, et al. The long noncoding RNA neuroLNC regulates presynaptic activity by interacting with the neurodegeneration-associated protein TDP-43. Sci Adv. 2019;5:eaay2670 pubmed publisher
|
- proximity ligation assay; human; loading ...; fig 2b
- immunocytochemistry; human; loading ...; fig 2d
| Wheeler J, McMillan P, Strovas T, Liachko N, Amlie Wolf A, Kow R, et al. Activity of the poly(A) binding protein MSUT2 determines susceptibility to pathological tau in the mammalian brain. Sci Transl Med. 2019;11: pubmed publisher
|
- immunohistochemistry - free floating section; mouse; 1:200; loading ...; fig 5
- western blot; mouse; 1:1000; loading ...; fig 7a
| Duan W, Guo M, Yi L, Zhang J, Bi Y, Liu Y, et al. Deletion of Tbk1 disrupts autophagy and reproduces behavioral and locomotor symptoms of FTD-ALS in mice. Aging (Albany NY). 2019;11:2457-2476 pubmed publisher
|
- western blot; fruit fly ; 1:1000; loading ...; fig 2b
| Berson A, Goodman L, Sartoris A, Otte C, Aykit J, Lee V, et al. Drosophila Ref1/ALYREF regulates transcription and toxicity associated with ALS/FTD disease etiologies. Acta Neuropathol Commun. 2019;7:65 pubmed publisher
|
- immunohistochemistry - paraffin section; human; 1:5000; loading ...; fig s8
- immunocytochemistry; human; 1:1000; loading ...; fig 1e
- western blot; human; 1:1000; loading ...; fig 1d
| Melamed Z, López Erauskin J, Baughn M, Zhang O, Drenner K, Sun Y, et al. Premature polyadenylation-mediated loss of stathmin-2 is a hallmark of TDP-43-dependent neurodegeneration. Nat Neurosci. 2019;22:180-190 pubmed publisher
|
- immunohistochemistry - paraffin section; mouse; loading ...; fig 9c
| Kuliyev E, Gingras S, Guy C, Howell S, Vogel P, Pelletier S. Overlapping Role of SCYL1 and SCYL3 in Maintaining Motor Neuron Viability. J Neurosci. 2018;38:2615-2630 pubmed publisher
|
- immunocytochemistry; rat; loading ...; fig 8a
- western blot; rat; 1:1000; loading ...; fig 8b
| Gemechu J, Sharma A, Yu D, Xie Y, Merkel O, Moszczynska A. Characterization of Dopaminergic System in the Striatum of Young Adult Park2-/- Knockout Rats. Sci Rep. 2018;8:1517 pubmed publisher
|
- immunocytochemistry; rat; loading ...; fig 1a
| Herzog J, Deshpande M, Shapiro L, Rodal A, Paradis S. TDP-43 misexpression causes defects in dendritic growth. Sci Rep. 2017;7:15656 pubmed publisher
|
- immunohistochemistry; human; 1:100; loading ...; fig s3b
| Krug L, Chatterjee N, Borges Monroy R, Hearn S, Liao W, Morrill K, et al. Retrotransposon activation contributes to neurodegeneration in a Drosophila TDP-43 model of ALS. PLoS Genet. 2017;13:e1006635 pubmed publisher
|
- immunocytochemistry; human; 1:500; loading ...; fig 3a
- western blot; human; 1:5000; loading ...; fig 4a
| Wobst H, Wesolowski S, Chadchankar J, Delsing L, Jacobsen S, Mukherjee J, et al. Cytoplasmic Relocalization of TAR DNA-Binding Protein 43 Is Not Sufficient to Reproduce Cellular Pathologies Associated with ALS In vitro. Front Mol Neurosci. 2017;10:46 pubmed publisher
|
- immunocytochemistry; human; fig s9d
| Shadle S, Zhong J, Campbell A, Conerly M, Jagannathan S, Wong C, et al. DUX4-induced dsRNA and MYC mRNA stabilization activate apoptotic pathways in human cell models of facioscapulohumeral dystrophy. PLoS Genet. 2017;13:e1006658 pubmed publisher
|
- immunocytochemistry; human; loading ...; fig 5a
| Lee K, Zhang P, Kim H, Mitrea D, Sarkar M, Freibaum B, et al. C9orf72 Dipeptide Repeats Impair the Assembly, Dynamics, and Function of Membrane-Less Organelles. Cell. 2016;167:774-788.e17 pubmed publisher
|
- immunocytochemistry; human; 1:500; loading ...; fig 5c
| Smethurst P, Newcombe J, Troakes C, Simone R, Chen Y, Patani R, et al. In vitro prion-like behaviour of TDP-43 in ALS. Neurobiol Dis. 2016;96:236-247 pubmed publisher
|
- western blot; human; 1:2000; fig 6d
| Kapeli K, Pratt G, Vu A, Hutt K, Martinez F, Sundararaman B, et al. Distinct and shared functions of ALS-associated proteins TDP-43, FUS and TAF15 revealed by multisystem analyses. Nat Commun. 2016;7:12143 pubmed publisher
|
- immunoprecipitation; mouse; fig 1
- western blot; mouse; 1:1000; fig 1
| Li R, Dong Q, Yuan X, Zeng X, Gao Y, Chiao C, et al. Misregulation of Alternative Splicing in a Mouse Model of Rett Syndrome. PLoS Genet. 2016;12:e1006129 pubmed publisher
|
- immunohistochemistry; mouse; 1:100
| Vernay A, Therreau L, Blot B, Risson V, Dirrig Grosch S, Waegaert R, et al. A transgenic mouse expressing CHMP2Bintron5 mutant in neurons develops histological and behavioural features of amyotrophic lateral sclerosis and frontotemporal dementia. Hum Mol Genet. 2016;25:3341-3360 pubmed publisher
|
- immunohistochemistry - paraffin section; human; fig 1
| Ando K, Tomimura K, Sazdovitch V, Suain V, Yilmaz Z, Authelet M, et al. Level of PICALM, a key component of clathrin-mediated endocytosis, is correlated with levels of phosphotau and autophagy-related proteins and is associated with tau inclusions in AD, PSP and Pick disease. Neurobiol Dis. 2016;94:32-43 pubmed publisher
|
- western blot; human; 1:500; fig 5
| Onesto E, Colombrita C, Gumina V, Borghi M, Dusi S, Doretti A, et al. Gene-specific mitochondria dysfunctions in human TARDBP and C9ORF72 fibroblasts. Acta Neuropathol Commun. 2016;4:47 pubmed publisher
|
- immunocytochemistry; human; fig 8
- western blot; human; fig 3
| Alquezar C, Salado I, de la Encarnación A, Perez D, Moreno F, Gil C, et al. Targeting TDP-43 phosphorylation by Casein Kinase-1? inhibitors: a novel strategy for the treatment of frontotemporal dementia. Mol Neurodegener. 2016;11:36 pubmed publisher
|
- immunohistochemistry - paraffin section; mouse; loading ...; fig s3g
| O Rourke J, Bogdanik L, Yáñez A, Lall D, Wolf A, Muhammad A, et al. C9orf72 is required for proper macrophage and microglial function in mice. Science. 2016;351:1324-9 pubmed publisher
|
- western blot; mouse; 1:10,000; fig 6
| Sharma A, Lyashchenko A, Lu L, Nasrabady S, Elmaleh M, Mendelsohn M, et al. ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function. Nat Commun. 2016;7:10465 pubmed publisher
|
- western blot; brewer's yeast; fig 3
| Torrente M, Chuang E, Noll M, Jackrel M, Go M, Shorter J. Mechanistic Insights into Hsp104 Potentiation. J Biol Chem. 2016;291:5101-15 pubmed publisher
|
| Venkataraman L, He P, Khan G, Harris B, Sierks M. Isolation and characterization of antibody fragments selective for human FTD brain derived TDP-43 variants. BMC Neurosci. 2020;21:36 pubmed publisher
|
| Neumann M, Frick P, Paron F, Kosten J, Buratti E, Mackenzie I. Antibody against TDP-43 phosphorylated at serine 375 suggests conformational differences of TDP-43 aggregates among FTLD-TDP subtypes. Acta Neuropathol. 2020;: pubmed publisher
|
| Bajc Česnik A, Motaln H, Rogelj B. The Impact of ALS-Associated Genes hnRNPA1, MATR3, VCP and UBQLN2 on the Severity of TDP-43 Aggregation. Cells. 2020;9: pubmed publisher
|
| Briese M, Saal Bauernschubert L, Lüningschrör P, Moradi M, Dombert B, Surrey V, et al. Loss of Tdp-43 disrupts the axonal transcriptome of motoneurons accompanied by impaired axonal translation and mitochondria function. Acta Neuropathol Commun. 2020;8:116 pubmed publisher
|
| Zhu Y, Liu Y, Yang F, Chen W, Jiang J, He P, et al. All-Trans Retinoic Acid Exerts Neuroprotective Effects in Amyotrophic Lateral Sclerosis-Like Tg (SOD1*G93A)1Gur Mice. Mol Neurobiol. 2020;57:3603-3615 pubmed publisher
|
| Wu H, Sun H, He Z, Chen X, Li Y, Zhao X, et al. The effect and mechanism of 19S proteasome PSMD11/Rpn6 subunit in D-Galactose induced mimetic aging models. Exp Cell Res. 2020;394:112093 pubmed publisher
|
| Sanna S, Esposito S, Masala A, Sini P, Nieddu G, Galioto M, et al. HDAC1 inhibition ameliorates TDP-43-induced cell death in vitro and in vivo. Cell Death Dis. 2020;11:369 pubmed publisher
|
| Tasca G, Lattante S, Marangi G, Conte A, Bernardo D, Bisogni G, et al. SOD1 p.D12Y variant is associated with amyotrophic lateral sclerosis/distal myopathy spectrum. Eur J Neurol. 2020;27:1304-1309 pubmed publisher
|
| Dobson Stone C, Hallupp M, Shahheydari H, Ragagnin A, Chatterton Z, Carew Jones F, et al. CYLD is a causative gene for frontotemporal dementia - amyotrophic lateral sclerosis. Brain. 2020;143:783-799 pubmed publisher
|
| Martínez González L, Rodríguez Cueto C, Cabezudo D, Bartolomé F, Andrés Benito P, Ferrer I, et al. Motor neuron preservation and decrease of in vivo TDP-43 phosphorylation by protein CK-1δ kinase inhibitor treatment. Sci Rep. 2020;10:4449 pubmed publisher
|
| Ormeño F, Hormazabal J, Moreno J, Riquelme F, Rios J, Criollo A, et al. Chaperone Mediated Autophagy Degrades TDP-43 Protein and Is Affected by TDP-43 Aggregation. Front Mol Neurosci. 2020;13:19 pubmed publisher
|
| Huang S, Wu L, Lee M, Chang C, Cheng W, Fang Y, et al. A robust TDP-43 knock-in mouse model of ALS. Acta Neuropathol Commun. 2020;8:3 pubmed publisher
|
| Zhao L, Ke H, Xu H, Wang G, Zhang H, Zou L, et al. TDP-43 facilitates milk lipid secretion by post-transcriptional regulation of Btn1a1 and Xdh. Nat Commun. 2020;11:341 pubmed publisher
|
| Braak H, Del Tredici K. From the Entorhinal Region via the Prosubiculum to the Dentate Fascia: Alzheimer Disease-Related Neurofibrillary Changes in the Temporal Allocortex. J Neuropathol Exp Neurol. 2020;79:163-175 pubmed publisher
|
| Sweeny E, Tariq A, Gurpinar E, Go M, Sochor M, Kan Z, et al. Structural and mechanistic insights into Hsp104 function revealed by synchrotron X-ray footprinting. J Biol Chem. 2020;295:1517-1538 pubmed publisher
|
| Nguyen T, Kabotyanski E, Reineke L, Shao J, Xiong F, Lee J, et al. The SINEB1 element in the long non-coding RNA Malat1 is necessary for TDP-43 proteostasis. Nucleic Acids Res. 2020;48:2621-2642 pubmed publisher
|
| Zhou Q, Mareljic N, Michaelsen M, Parhizkar S, Heindl S, Nuscher B, et al. Active poly-GA vaccination prevents microglia activation and motor deficits in a C9orf72 mouse model. EMBO Mol Med. 2020;12:e10919 pubmed publisher
|
| Isobe M, Toya H, Mito M, Chiba T, Asahara H, Hirose T, et al. Forced isoform switching of Neat1_1 to Neat1_2 leads to the loss of Neat1_1 and the hyperformation of paraspeckles but does not affect the development and growth of mice. RNA. 2020;26:251-264 pubmed publisher
|
| Robinson A, Chew Graham S, Davidson Y, Horan M, Roncaroli F, Minshull J, et al. A Comparative Study of Pathological Outcomes in The University of Manchester Longitudinal Study of Cognition in Normal Healthy Old Age and Brains for Dementia Research Cohorts. J Alzheimers Dis. 2020;73:619-632 pubmed publisher
|
| Hans F, Glasebach H, Kahle P. Multiple distinct pathways lead to hyperubiquitylated insoluble TDP-43 protein independent of its translocation into stress granules. J Biol Chem. 2020;295:673-689 pubmed publisher
|
| Zhao C, Devlin A, Chouhan A, Selvaraj B, Stavrou M, Burr K, et al. Mutant C9orf72 human iPSC-derived astrocytes cause non-cell autonomous motor neuron pathophysiology. Glia. 2019;: pubmed publisher
|
| Thierry M, Boluda S, Delatour B, Marty S, Seilhean D, Potier M, et al. Human subiculo-fornico-mamillary system in Alzheimer's disease: Tau seeding by the pillar of the fornix. Acta Neuropathol. 2019;: pubmed publisher
|
| Klingstedt T, Ghetti B, Holton J, Ling H, Nilsson K, Goedert M. Luminescent conjugated oligothiophenes distinguish between α-synuclein assemblies of Parkinson's disease and multiple system atrophy. Acta Neuropathol Commun. 2019;7:193 pubmed publisher
|
| Maurel C, Chami A, Thepault R, Marouillat S, Blasco H, Corcia P, et al. A role for SUMOylation in the Formation and Cellular Localization of TDP-43 Aggregates in Amyotrophic Lateral Sclerosis. Mol Neurobiol. 2019;: pubmed publisher
|
| Quadri Z, Johnson N, Zamudio F, Miller A, Peters M, Smeltzer S, et al. Overexpression of human wtTDP-43 causes impairment in hippocampal plasticity and behavioral deficits in CAMKII-tTa transgenic mouse model. Mol Cell Neurosci. 2019;102:103418 pubmed publisher
|
| Chadchankar J, Korboukh V, Conway L, Wobst H, Walker C, Doig P, et al. Inactive USP14 and inactive UCHL5 cause accumulation of distinct ubiquitinated proteins in mammalian cells. PLoS ONE. 2019;14:e0225145 pubmed publisher
|
| Couly S, Khalil B, Viguier V, Roussel J, Maurice T, Liévens J. Sigma-1 receptor is a key genetic modulator in amyotrophic lateral sclerosis. Hum Mol Genet. 2019;: pubmed publisher
|
| Kawaguchi T, Rollins M, Moinpour M, MORERA A, Ebmeier C, Old W, et al. Changes to the TDP-43 and FUS Interactomes Induced by DNA Damage. J Proteome Res. 2019;: pubmed publisher
|
| Lee S, Jeon Y, Cha S, Kim S, Kwon Y, Jo M, et al. PTK2/FAK regulates UPS impairment via SQSTM1/p62 phosphorylation in TARDBP/TDP-43 proteinopathies. Autophagy. 2019;:1-17 pubmed publisher
|
| Garces M, Guijarro M, Vargas A, Badiola J, Monzon M. Neuroglial patterns are shared by cerebella from prion and prion-like disorder affected patients. Mech Ageing Dev. 2019;184:111176 pubmed publisher
|
| Tam O, Rozhkov N, Shaw R, Kim D, Hubbard I, Fennessey S, et al. Postmortem Cortex Samples Identify Distinct Molecular Subtypes of ALS: Retrotransposon Activation, Oxidative Stress, and Activated Glia. Cell Rep. 2019;29:1164-1177.e5 pubmed publisher
|
| Bizarro J, Bhardwaj A, Smith S, Meier U. Nopp140-mediated concentration of telomerase in Cajal bodies regulates telomere length. Mol Biol Cell. 2019;30:3136-3150 pubmed publisher
|
| MORERA A, Ahmed N, Schwartz J. TDP-43 regulates transcription at protein-coding genes and Alu retrotransposons. Biochim Biophys Acta Gene Regul Mech. 2019;1862:194434 pubmed publisher
|
| Schmidt H, Barreau A, Rohatgi R. Phase separation-deficient TDP43 remains functional in splicing. Nat Commun. 2019;10:4890 pubmed publisher
|
| Williamson M, Finelli M, Sleigh J, Reddington A, Gordon D, Talbot K, et al. Neuronal over-expression of Oxr1 is protective against ALS-associated mutant TDP-43 mislocalisation in motor neurons and neuromuscular defects in vivo. Hum Mol Genet. 2019;28:3584-3599 pubmed publisher
|
| Zhu J, Wang N, Li X, Zheng X, Zhao J, Xia H, et al. Suppression of Progranulin Expression Leads to Formation of Intranuclear TDP-43 Inclusions In Vitro: A Cell Model of Frontotemporal Lobar Degeneration. J Neuropathol Exp Neurol. 2019;78:1124-1129 pubmed publisher
|
| Brettle M, Stefen H, Djordjevic A, Fok S, Chan J, Van Hummel A, et al. Developmental Expression of Mutant PFN1 in Motor Neurons Impacts Neuronal Growth and Motor Performance of Young and Adult Mice. Front Mol Neurosci. 2019;12:231 pubmed publisher
|
| Chen H, Topp S, Hui H, Zacco E, Katarya M, McLoughlin C, et al. RRM adjacent TARDBP mutations disrupt RNA binding and enhance TDP-43 proteinopathy. Brain. 2019;142:3753-3770 pubmed publisher
|
| Mori S, Honda H, Ishii T, Yoshimura M, Sasagasako N, Suzuki S, et al. Expanded polyglutamine impairs normal nuclear distribution of fused in sarcoma and poly (rC)-binding protein 1 in Huntington's disease. Neuropathology. 2019;39:358-367 pubmed publisher
|
| Chen J, Nathaniel D, Raghavan P, Nelson M, Tian R, Tse E, et al. Compromised function of the ESCRT pathway promotes endolysosomal escape of tau seeds and propagation of tau aggregation. J Biol Chem. 2019;294:18952-18966 pubmed publisher
|
| Liao Y, Fernandopulle M, Wang G, Choi H, Hao L, Drerup C, et al. RNA Granules Hitchhike on Lysosomes for Long-Distance Transport, Using Annexin A11 as a Molecular Tether. Cell. 2019;179:147-164.e20 pubmed publisher
|
| Yamashita S, Kimura E, Zhang Z, Tawara N, Hara K, Yoshimura A, et al. Muscle pathology of hereditary motor and sensory neuropathy with proximal dominant involvement with TFG mutation. Muscle Nerve. 2019;: pubmed publisher
|
| Tariq A, Lin J, Jackrel M, Hesketh C, Carman P, Mack K, et al. Mining Disaggregase Sequence Space to Safely Counter TDP-43, FUS, and α-Synuclein Proteotoxicity. Cell Rep. 2019;28:2080-2095.e6 pubmed publisher
|
| Heyburn L, Abutarboush R, Goodrich S, Urioste R, Batuure A, Statz J, et al. Repeated Low-Level Blast Overpressure Leads to Endovascular Disruption and Alterations in TDP-43 and Piezo2 in a Rat Model of Blast TBI. Front Neurol. 2019;10:766 pubmed publisher
|
| Yamashita S, Matsuo Y, Tawara N, Hara K, Yamamoto M, Nishikami T, et al. CYLD dysregulation in pathogenesis of sporadic inclusion body myositis. Sci Rep. 2019;9:11606 pubmed publisher
|
| Fischer N, Preuße C, Radke J, Pehl D, Allenbach Y, Schneider U, et al. Sequestosome-1 (p62) expression reveals chaperone-assisted selective autophagy in immune-mediated necrotizing myopathies. Brain Pathol. 2019;: pubmed publisher
|
| Gu J, Hu W, Tan X, Qu S, Chu D, Gong C, et al. Elevation of casein kinase 1ε associated with TDP-43 and tau pathologies in Alzheimer's disease. Brain Pathol. 2019;: pubmed publisher
|
| Donde A, Sun M, Ling J, Braunstein K, Pang B, Wen X, et al. Splicing repression is a major function of TDP-43 in motor neurons. Acta Neuropathol. 2019;: pubmed publisher
|
| Sugai A, Kato T, Koyama A, Koike Y, Konno T, Ishihara T, et al. Non-genetically modified models exhibit TARDBP mRNA increase due to perturbed TDP-43 autoregulation. Neurobiol Dis. 2019;130:104534 pubmed publisher
|
| Shi Y, Hung S, Rocha G, Lin S, Linares G, Staats K, et al. Identification and therapeutic rescue of autophagosome and glutamate receptor defects in C9ORF72 and sporadic ALS neurons. JCI Insight. 2019;5: pubmed publisher
|
| Kim S, Chung A, Na J, Lee S, Jeong S, Kim E, et al. Myelin degeneration induced by mutant superoxide dismutase 1 accumulation promotes amyotrophic lateral sclerosis. Glia. 2019;67:1910-1921 pubmed publisher
|
| Hicks D, Cross L, Williamson R, Rattray M. Endoplasmic Reticulum Stress Signalling Induces Casein Kinase 1-Dependent Formation of Cytosolic TDP-43 Inclusions in Motor Neuron-Like Cells. Neurochem Res. 2019;: pubmed publisher
|
| Hao Z, Liu L, Tao Z, Wang R, Ren H, Sun H, et al. Motor dysfunction and neurodegeneration in a C9orf72 mouse line expressing poly-PR. Nat Commun. 2019;10:2906 pubmed publisher
|
| Josephs K, Zhang Y, Baker M, Rademakers R, Petrucelli L, Dickson D. C-terminal and full length TDP-43 specie differ according to FTLD-TDP lesion type but not genetic mutation. Acta Neuropathol Commun. 2019;7:100 pubmed publisher
|
| Donde A, Sun M, Jeong Y, Wen X, Ling J, Lin S, et al. Upregulation of ATG7 attenuates motor neuron dysfunction associated with depletion of TARDBP/TDP-43. Autophagy. 2019;:1-11 pubmed publisher
|
| Larocca T, Mariani A, Watkins L, Link C. TDP-43 knockdown causes innate immune activation via protein kinase R in astrocytes. Neurobiol Dis. 2019;:104514 pubmed publisher
|
| Kattuah W, Rogelj B, King A, Shaw C, Hortobagyi T, Troakes C. Heterogeneous Nuclear Ribonucleoprotein E2 (hnRNP E2) Is a Component of TDP-43 Aggregates Specifically in the A and C Pathological Subtypes of Frontotemporal Lobar Degeneration. Front Neurosci. 2019;13:551 pubmed publisher
|
| Koza P, Beroun A, Konopka A, Gorkiewicz T, Bijoch L, Torres J, et al. Neuronal TDP-43 depletion affects activity-dependent plasticity. Neurobiol Dis. 2019;130:104499 pubmed publisher
|
| Nicoll J, Buckland G, Harrison C, Page A, Harris S, Love S, et al. Persistent neuropathological effects 14 years following amyloid-β immunization in Alzheimer's disease. Brain. 2019;: pubmed publisher
|
| Riku Y, Duyckaerts C, Boluda S, Plu I, Le Ber I, Millecamps S, et al. Increased prevalence of granulovacuolar degeneration in C9orf72 mutation. Acta Neuropathol. 2019;: pubmed publisher
|
| Takahashi Y, Uchino A, Shioya A, Sano T, Matsumoto C, Numata Uematsu Y, et al. Altered immunoreactivity of ErbB4, a causative gene product for ALS19, in the spinal cord of patients with sporadic ALS. Neuropathology. 2019;39:268-278 pubmed publisher
|
| Goodman L, Prudencio M, Kramer N, Martinez Ramirez L, Srinivasan A, Lan M, et al. Toxic expanded GGGGCC repeat transcription is mediated by the PAF1 complex in C9orf72-associated FTD. Nat Neurosci. 2019;: pubmed publisher
|
| Wang P, Deng J, Dong J, Liu J, Bigio E, Mesulam M, et al. TDP-43 induces mitochondrial damage and activates the mitochondrial unfolded protein response. PLoS Genet. 2019;15:e1007947 pubmed publisher
|
| Gregory A, Lotia M, Jeong S, Fox R, Zhen D, Sanford L, et al. Autosomal dominant mitochondrial membrane protein-associated neurodegeneration (MPAN). Mol Genet Genomic Med. 2019;:e736 pubmed publisher
|
| Modic M, Grosch M, Rot G, Schirge S, Lepko T, Yamazaki T, et al. Cross-Regulation between TDP-43 and Paraspeckles Promotes Pluripotency-Differentiation Transition. Mol Cell. 2019;74:951-965.e13 pubmed publisher
|
| Liu E, Russ J, Cali C, Phan J, Amlie Wolf A, Lee E. Loss of Nuclear TDP-43 Is Associated with Decondensation of LINE Retrotransposons. Cell Rep. 2019;27:1409-1421.e6 pubmed publisher
|
| Naasan G, Shany Ur T, Sidhu M, Barton C, Ketelle R, Shdo S, et al. Corticobasal syndrome with visual hallucinations and probable REM-sleep behavior disorder: an autopsied case report of a patient with CBD and LBD pathology. Neurocase. 2019;25:26-33 pubmed publisher
|
| Wang B, Maxwell B, Joo J, Gwon Y, Messing J, Mishra A, et al. ULK1 and ULK2 Regulate Stress Granule Disassembly Through Phosphorylation and Activation of VCP/p97. Mol Cell. 2019;74:742-757.e8 pubmed publisher
|
| Gasset Rosa F, Lu S, Yu H, Chen C, Melamed Z, Guo L, et al. Cytoplasmic TDP-43 De-mixing Independent of Stress Granules Drives Inhibition of Nuclear Import, Loss of Nuclear TDP-43, and Cell Death. Neuron. 2019;102:339-357.e7 pubmed publisher
|
| Banerjee A, Phillips B, Deng Q, Seyfried N, Pavlath G, Vest K, et al. Proteomic analysis reveals that wildtype and alanine-expanded nuclear poly(A)-binding protein exhibit differential interactions in skeletal muscle. J Biol Chem. 2019;294:7360-7376 pubmed publisher
|
| Mann J, Gleixner A, Mauna J, Gomes E, DeChellis Marks M, Needham P, et al. RNA Binding Antagonizes Neurotoxic Phase Transitions of TDP-43. Neuron. 2019;102:321-338.e8 pubmed publisher
|
| French R, Grese Z, Aligireddy H, Dhavale D, Reeb A, Kedia N, et al. Detection of TAR DNA-binding protein 43 (TDP-43) oligomers as initial intermediate species during aggregate formation. J Biol Chem. 2019;294:6696-6709 pubmed publisher
|
| Horos R, Büscher M, Kleinendorst R, Alleaume A, Tarafder A, Schwarzl T, et al. The Small Non-coding Vault RNA1-1 Acts as a Riboregulator of Autophagy. Cell. 2019;176:1054-1067.e12 pubmed publisher
|
| Ling S, Dastidar S, Tokunaga S, Ho W, Lim K, Ilieva H, et al. Overriding FUS autoregulation in mice triggers gain-of-toxic dysfunctions in RNA metabolism and autophagy-lysosome axis. elife. 2019;8: pubmed publisher
|
| Huntley M, Gao J, Termsarasab P, Wang L, Zeng S, Thammongkolchai T, et al. Association between TDP-43 and mitochondria in inclusion body myositis. Lab Invest. 2019;: pubmed publisher
|
| Tsai R, Bejanin A, Lesman Segev O, LaJoie R, Visani A, Bourakova V, et al. 18F-flortaucipir (AV-1451) tau PET in frontotemporal dementia syndromes. Alzheimers Res Ther. 2019;11:13 pubmed publisher
|
| Elia L, Mason A, Alijagic A, Finkbeiner S. Genetic Regulation of Neuronal Progranulin Reveals a Critical Role for the Autophagy-Lysosome Pathway. J Neurosci. 2019;39:3332-3344 pubmed publisher
|
| Pozzi S, Thammisetty S, Codron P, Rahimian R, Plourde K, Soucy G, et al. Virus-mediated delivery of antibody targeting TAR DNA-binding protein-43 mitigates associated neuropathology. J Clin Invest. 2019;129:1581-1595 pubmed publisher
|
| Arhzaouy K, Papadopoulos C, Schulze N, Pittman S, Meyer H, Weihl C. VCP maintains lysosomal homeostasis and TFEB activity in differentiated skeletal muscle. Autophagy. 2019;:1-18 pubmed publisher
|
| Klim J, Williams L, Limone F, Guerra San Juan I, Davis Dusenbery B, Mordes D, et al. ALS-implicated protein TDP-43 sustains levels of STMN2, a mediator of motor neuron growth and repair. Nat Neurosci. 2019;22:167-179 pubmed publisher
|
| Chen Y, Cohen T. Aggregation of the nucleic acid-binding protein TDP-43 occurs via distinct routes that are coordinated with stress granule formation. J Biol Chem. 2019;294:3696-3706 pubmed publisher
|
| Ebstein S, Yagudayeva I, Shneider N. Mutant TDP-43 Causes Early-Stage Dose-Dependent Motor Neuron Degeneration in a TARDBP Knockin Mouse Model of ALS. Cell Rep. 2019;26:364-373.e4 pubmed publisher
|
| Aizawa H, Yamashita T, Kato H, Kimura T, Kwak S. Impaired Nucleoporins Are Present in Sporadic Amyotrophic Lateral Sclerosis Motor Neurons that Exhibit Mislocalization of the 43-kDa TAR DNA-Binding Protein. J Clin Neurol. 2019;15:62-67 pubmed publisher
|
| Michalska K, Zhang K, March Z, Hatzos Skintges C, Pintilie G, Bigelow L, et al. Structure of Calcarisporiella thermophila Hsp104 Disaggregase that Antagonizes Diverse Proteotoxic Misfolding Events. Structure. 2019;27:449-463.e7 pubmed publisher
|
| Hishizawa M, Yamashita H, Akizuki M, Urushitani M, Takahashi R. TDP-43 levels are higher in platelets from patients with sporadic amyotrophic lateral sclerosis than in healthy controls. Neurochem Int. 2019;124:41-45 pubmed publisher
|
| Laferrière F, Maniecka Z, Pérez Berlanga M, Hruska Plochan M, Gilhespy L, Hock E, et al. TDP-43 extracted from frontotemporal lobar degeneration subject brains displays distinct aggregate assemblies and neurotoxic effects reflecting disease progression rates. Nat Neurosci. 2019;22:65-77 pubmed publisher
|
| Sun Y, Medina Cruz A, Hadley K, Galant N, Law R, Vernon R, et al. Physiologically Important Electrolytes as Regulators of TDP-43 Aggregation and Droplet-Phase Behavior. Biochemistry. 2019;58:590-607 pubmed publisher
|
| Crociara P, Chieppa M, Vallino Costassa E, Berrone E, Gallo M, Lo Faro M, et al. Motor neuron degeneration, severe myopathy and TDP-43 increase in a transgenic pig model of SOD1-linked familiar ALS. Neurobiol Dis. 2019;124:263-275 pubmed publisher
|
| Steinacker P, Barschke P, Otto M. Biomarkers for diseases with TDP-43 pathology. Mol Cell Neurosci. 2019;97:43-59 pubmed publisher
|
| Fourier A, Escal J, Bernard E, Lachman I, Perret Liaudet A, Leblanc P, et al. Development of an automated capillary nano-immunoassay-Simple Western assay-to quantify total TDP43 protein in human platelet samples. Anal Bioanal Chem. 2019;411:267-275 pubmed publisher
|
| Gordon D, Dafinca R, Scaber J, Alegre Abarrategui J, Farrimond L, Scott C, et al. Single-copy expression of an amyotrophic lateral sclerosis-linked TDP-43 mutation (M337V) in BAC transgenic mice leads to altered stress granule dynamics and progressive motor dysfunction. Neurobiol Dis. 2019;121:148-162 pubmed publisher
|
| Statland J, Moore D, Wang Y, Walsh M, Mozaffar T, Elman L, et al. Rasagiline for amyotrophic lateral sclerosis: A randomized, controlled trial. Muscle Nerve. 2019;59:201-207 pubmed publisher
|
| Akinyemi R, Salami A, Akinyemi J, Ojagbemi A, Olopade F, Coker M, et al. Brain banking in low and middle-income countries: Raison D'être for the Ibadan Brain Ageing, Dementia And Neurodegeneration (IBADAN) Brain Bank Project. Brain Res Bull. 2019;145:136-141 pubmed publisher
|
| Jawaid A, Woldemichael B, Kremer E, Laferrière F, Gaur N, Afroz T, et al. Memory Decline and Its Reversal in Aging and Neurodegeneration Involve miR-183/96/182 Biogenesis. Mol Neurobiol. 2019;56:3451-3462 pubmed publisher
|
| Posa D, Martínez González L, Bartolomé F, Nagaraj S, Porras G, Martinez A, et al. Recapitulation of Pathological TDP-43 Features in Immortalized Lymphocytes from Sporadic ALS Patients. Mol Neurobiol. 2019;56:2424-2432 pubmed publisher
|
| Jeon G, Shim Y, Lee D, Kim J, Kang M, Ahn S, et al. Pathological Modification of TDP-43 in Amyotrophic Lateral Sclerosis with SOD1 Mutations. Mol Neurobiol. 2019;56:2007-2021 pubmed publisher
|
| Kim J, Billington E, Reyes A, Notarianni T, Sage J, Agbas E, et al. Impaired Cu-Zn Superoxide Dismutase (SOD1) and Calcineurin (Cn) Interaction in ALS: A Presumed Consequence for TDP-43 and Zinc Aggregation in Tg SOD1G93A Rodent Spinal Cord Tissue. Neurochem Res. 2019;44:228-233 pubmed publisher
|
| Katsumata Y, Fardo D, Kukull W, Nelson P. Dichotomous scoring of TDP-43 proteinopathy from specific brain regions in 27 academic research centers: associations with Alzheimer's disease and cerebrovascular disease pathologies. Acta Neuropathol Commun. 2018;6:142 pubmed publisher
|
| Pereira G, Sanchez L, Schaughency P, Rubio Roldán A, Choi J, Planet E, et al. Properties of LINE-1 proteins and repeat element expression in the context of amyotrophic lateral sclerosis. Mob DNA. 2018;9:35 pubmed publisher
|
| Pons M, Prieto S, Miguel L, Frebourg T, Campion D, Suñé C, et al. Identification of TCERG1 as a new genetic modulator of TDP-43 production in Drosophila. Acta Neuropathol Commun. 2018;6:138 pubmed publisher
|
| Nana A, Sidhu M, Gaus S, Hwang J, Li L, Park Y, et al. Neurons selectively targeted in frontotemporal dementia reveal early stage TDP-43 pathobiology. Acta Neuropathol. 2018;: pubmed publisher
|
| Shin Y, Chang Y, Lee D, Berry J, Sanders D, Ronceray P, et al. Liquid Nuclear Condensates Mechanically Sense and Restructure the Genome. Cell. 2018;175:1481-1491.e13 pubmed publisher
|
| Thompson V, Victor R, MORERA A, Moinpour M, Liu M, Kisiel C, et al. Transcription-Dependent Formation of Nuclear Granules Containing FUS and RNA Pol II. Biochemistry. 2018;57:7021-7032 pubmed publisher
|
| Cai H, Tian K, Zhang Y, Jiang H, Han S. Angiopoietin-1 and ανβ3 integrin peptide promote the therapeutic effects of L-serine in an amyotrophic lateral sclerosis/Parkinsonism dementia complex model. Aging (Albany NY). 2018;10:3507-3527 pubmed publisher
|
| Vogler T, Wheeler J, Nguyen E, Hughes M, Britson K, Lester E, et al. TDP-43 and RNA form amyloid-like myo-granules in regenerating muscle. Nature. 2018;563:508-513 pubmed publisher
|
| Thammisetty S, Pedragosa J, Weng Y, Calon F, Planas A, Kriz J. Age-related deregulation of TDP-43 after stroke enhances NF-κB-mediated inflammation and neuronal damage. J Neuroinflammation. 2018;15:312 pubmed publisher
|
| Chen T, Huang B, Shi X, Gao L, Huang C. Mutant UBQLN2P497H in motor neurons leads to ALS-like phenotypes and defective autophagy in rats. Acta Neuropathol Commun. 2018;6:122 pubmed publisher
|
| Picher Martel V, Renaud L, Bareil C, Julien J. Neuronal Expression of UBQLN2P497H Exacerbates TDP-43 Pathology in TDP-43G348C Mice through Interaction with Ubiquitin. Mol Neurobiol. 2018;: pubmed publisher
|
| Chung C, Berson A, Kennerdell J, Sartoris A, Unger T, Porta S, et al. Aberrant activation of non-coding RNA targets of transcriptional elongation complexes contributes to TDP-43 toxicity. Nat Commun. 2018;9:4406 pubmed publisher
|
| Sharkey L, Safren N, Pithadia A, Gerson J, Dulchavsky M, Fischer S, et al. Mutant UBQLN2 promotes toxicity by modulating intrinsic self-assembly. Proc Natl Acad Sci U S A. 2018;115:E10495-E10504 pubmed publisher
|
| Beel S, Herdewyn S, Fazal R, De Decker M, Moisse M, Robberecht W, et al. Progranulin reduces insoluble TDP-43 levels, slows down axonal degeneration and prolongs survival in mutant TDP-43 mice. Mol Neurodegener. 2018;13:55 pubmed publisher
|
| Xue Y, Ruller C, Fung G, Mohamud Y, Deng H, Liu H, et al. Enteroviral Infection Leads to Transactive Response DNA-Binding Protein 43 Pathology in Vivo. Am J Pathol. 2018;188:2853-2862 pubmed publisher
|
| Sun X, Duan Y, Qin C, Li J, Duan G, Deng X, et al. Distinct multilevel misregulations of Parkin and PINK1 revealed in cell and animal models of TDP-43 proteinopathy. Cell Death Dis. 2018;9:953 pubmed publisher
|
| Paul S, Dansithong W, Figueroa K, Scoles D, Pulst S. Staufen1 links RNA stress granules and autophagy in a model of neurodegeneration. Nat Commun. 2018;9:3648 pubmed publisher
|
| Xu D, Jin T, Zhu H, Chen H, Ofengeim D, Zou C, et al. TBK1 Suppresses RIPK1-Driven Apoptosis and Inflammation during Development and in Aging. Cell. 2018;174:1477-1491.e19 pubmed publisher
|
| Masala A, Sanna S, Esposito S, Rassu M, Galioto M, Zinellu A, et al. Epigenetic Changes Associated with the Expression of Amyotrophic Lateral Sclerosis (ALS) Causing Genes. Neuroscience. 2018;390:1-11 pubmed publisher
|
| Tawara N, Yamashita S, Kawakami K, Kurashige T, Zhang Z, Tasaki M, et al. Muscle-dominant wild-type TDP-43 expression induces myopathological changes featuring tubular aggregates and TDP-43-positive inclusions. Exp Neurol. 2018;309:169-180 pubmed publisher
|
| Hans F, Eckert M, von Zweydorf F, Gloeckner C, Kahle P. Identification and characterization of ubiquitinylation sites in TAR DNA-binding protein of 43 kDa (TDP-43). J Biol Chem. 2018;293:16083-16099 pubmed publisher
|
| McGurk L, Gomes E, Guo L, Mojsilovic Petrovic J, Tran V, Kalb R, et al. Poly(ADP-Ribose) Prevents Pathological Phase Separation of TDP-43 by Promoting Liquid Demixing and Stress Granule Localization. Mol Cell. 2018;71:703-717.e9 pubmed publisher
|
| Ibba G, Piu C, Uleri E, Serra C, Dolei A. Disruption by SaCas9 Endonuclease of HERV-Kenv, a Retroviral Gene with Oncogenic and Neuropathogenic Potential, Inhibits Molecules Involved in Cancer and Amyotrophic Lateral Sclerosis. Viruses. 2018;10: pubmed publisher
|
| Conlon E, Fagegaltier D, Agius P, Davis Porada J, Gregory J, Hubbard I, et al. Unexpected similarities between C9ORF72 and sporadic forms of ALS/FTD suggest a common disease mechanism. elife. 2018;7: pubmed publisher
|
| Braak H, Del Tredici K. Spreading of Tau Pathology in Sporadic Alzheimer's Disease Along Cortico-cortical Top-Down Connections. Cereb Cortex. 2018;28:3372-3384 pubmed publisher
|
| Davidson Y, Robinson A, Prasher V, Mann D. The age of onset and evolution of Braak tangle stage and Thal amyloid pathology of Alzheimer's disease in individuals with Down syndrome. Acta Neuropathol Commun. 2018;6:56 pubmed publisher
|
| Boddaert J, Wils H, Kumar Singh S. Methods to Investigate the Molecular Basis of Progranulin Actions on Brain and Behavior In Vivo Using Knockout Mice. Methods Mol Biol. 2018;1806:233-253 pubmed publisher
|
| Narcís J, Tapia O, Tarabal O, Piedrafita L, Caldero J, Berciano M, et al. Accumulation of poly(A) RNA in nuclear granules enriched in Sam68 in motor neurons from the SMN?7 mouse model of SMA. Sci Rep. 2018;8:9646 pubmed publisher
|
| Torres P, Ramírez Nuñez O, Romero Guevara R, Barés G, Granado Serrano A, Ayala V, et al. Cryptic exon splicing function of TARDBP interacts with autophagy in nervous tissue. Autophagy. 2018;14:1398-1403 pubmed publisher
|
| Tan X, Sun M, Brady R, Liu S, Llanos R, Cheung S, et al. Transactive Response DNA-Binding Protein 43 Abnormalities after Traumatic Brain Injury. J Neurotrauma. 2018;: pubmed publisher
|
| Shelkovnikova T, Kukharsky M, An H, Dimasi P, Alexeeva S, Shabir O, et al. Protective paraspeckle hyper-assembly downstream of TDP-43 loss of function in amyotrophic lateral sclerosis. Mol Neurodegener. 2018;13:30 pubmed publisher
|
| Bossolasco P, Sassone F, Gumina V, Peverelli S, Garzo M, Silani V. Motor neuron differentiation of iPSCs obtained from peripheral blood of a mutant TARDBP ALS patient. Stem Cell Res. 2018;30:61-68 pubmed publisher
|
| Tariq A, Lin J, Noll M, Torrente M, Mack K, Murillo O, et al. Potentiating Hsp104 activity via phosphomimetic mutations in the middle domain. FEMS Yeast Res. 2018;18: pubmed publisher
|
| Scherz B, Rabl R, Flunkert S, Rohler S, Neddens J, Taub N, et al. mTh1 driven expression of hTDP-43 results in typical ALS/FTLD neuropathological symptoms. PLoS ONE. 2018;13:e0197674 pubmed publisher
|
| Miguel L, Avequin T, Pons M, Frebourg T, Campion D, Lecourtois M. FTLD/ALS-linked TDP-43 mutations do not alter TDP-43's ability to self-regulate its expression in Drosophila. Brain Res. 2018;1695:1-9 pubmed publisher
|
| Khalfallah Y, Kuta R, Grasmuck C, Prat A, Durham H, Vande Velde C. TDP-43 regulation of stress granule dynamics in neurodegenerative disease-relevant cell types. Sci Rep. 2018;8:7551 pubmed publisher
|
| Fratta P, Sivakumar P, Humphrey J, Lo K, Ricketts T, Oliveira H, et al. Mice with endogenous TDP-43 mutations exhibit gain of splicing function and characteristics of amyotrophic lateral sclerosis. EMBO J. 2018;37: pubmed publisher
|
| Hoffmann N, Weise S, Marinaro F, Vogel T, De Pietri Tonelli D. DGCR8 Promotes Neural Progenitor Expansion and Represses Neurogenesis in the Mouse Embryonic Neocortex. Front Neurosci. 2018;12:281 pubmed publisher
|
| Sun K, Li X, Chen X, Bai Y, Zhou G, Kokiko Cochran O, et al. Neuron-Specific HuR-Deficient Mice Spontaneously Develop Motor Neuron Disease. J Immunol. 2018;201:157-166 pubmed publisher
|
| Endo R, Takashima N, Nekooki Machida Y, Komi Y, Hui K, Takao M, et al. TAR DNA-Binding Protein 43 and Disrupted in Schizophrenia 1 Coaggregation Disrupts Dendritic Local Translation and Mental Function in Frontotemporal Lobar Degeneration. Biol Psychiatry. 2018;84:509-521 pubmed publisher
|
| Pinarbasi E, Cagatay T, Fung H, Li Y, Chook Y, Thomas P. Active nuclear import and passive nuclear export are the primary determinants of TDP-43 localization. Sci Rep. 2018;8:7083 pubmed publisher
|
| Koga S, Lin W, Walton R, Ross O, Dickson D. TDP-43 pathology in multiple system atrophy: colocalization of TDP-43 and α-synuclein in glial cytoplasmic inclusions. Neuropathol Appl Neurobiol. 2018;44:707-721 pubmed publisher
|
| Cykowski M, Powell S, Appel J, Arumanayagam A, Rivera A, Appel S. Phosphorylated TDP-43 (pTDP-43) aggregates in the axial skeletal muscle of patients with sporadic and familial amyotrophic lateral sclerosis. Acta Neuropathol Commun. 2018;6:28 pubmed publisher
|
| Niu Z, Pontifex C, Berini S, Hamilton L, Naddaf E, Wieben E, et al. Myopathy With SQSTM1 and TIA1 Variants: Clinical and Pathological Features. Front Neurol. 2018;9:147 pubmed publisher
|
| Deshaies J, Shkreta L, Moszczynski A, Sidibé H, Semmler S, Fouillen A, et al. TDP-43 regulates the alternative splicing of hnRNP A1 to yield an aggregation-prone variant in amyotrophic lateral sclerosis. Brain. 2018;141:1320-1333 pubmed publisher
|
| Wen J, Lv R, Ma H, Shen H, He C, Wang J, et al. Zc3h13 Regulates Nuclear RNA m6A Methylation and Mouse Embryonic Stem Cell Self-Renewal. Mol Cell. 2018;69:1028-1038.e6 pubmed publisher
|
| Archbold H, Jackson K, Arora A, Weskamp K, Tank E, Li X, et al. TDP43 nuclear export and neurodegeneration in models of amyotrophic lateral sclerosis and frontotemporal dementia. Sci Rep. 2018;8:4606 pubmed publisher
|
| Volkening K, Keller B, Leystra Lantz C, Strong M. RNA and Protein Interactors with TDP-43 in Human Spinal-Cord Lysates in Amyotrophic Lateral Sclerosis. J Proteome Res. 2018;17:1712-1729 pubmed publisher
|
| Nguyen A, Nguyen T, Zhang J, Devireddy S, Zhou P, Karydas A, et al. Murine knockin model for progranulin-deficient frontotemporal dementia with nonsense-mediated mRNA decay. Proc Natl Acad Sci U S A. 2018;115:E2849-E2858 pubmed publisher
|
| Honda H, Sasagasako N, Shen C, Shijo M, Hamasaki H, Suzuki S, et al. DCTN1 F52L mutation case of Perry syndrome with progressive supranuclear palsy-like tauopathy. Parkinsonism Relat Disord. 2018;51:105-110 pubmed publisher
|
| Feneberg E, Gray E, Ansorge O, Talbot K, Turner M. Towards a TDP-43-Based Biomarker for ALS and FTLD. Mol Neurobiol. 2018;55:7789-7801 pubmed publisher
|
| Wang A, Conicella A, Schmidt H, Martin E, Rhoads S, Reeb A, et al. A single N-terminal phosphomimic disrupts TDP-43 polymerization, phase separation, and RNA splicing. EMBO J. 2018;37: pubmed publisher
|
| Kahl A, Blanco I, Jackman K, Baskar J, Milaganur Mohan H, Rodney Sandy R, et al. Cerebral ischemia induces the aggregation of proteins linked to neurodegenerative diseases. Sci Rep. 2018;8:2701 pubmed publisher
|
| Tanikawa C, Ueda K, Suzuki A, Iida A, Nakamura R, Atsuta N, et al. Citrullination of RGG Motifs in FET Proteins by PAD4 Regulates Protein Aggregation and ALS Susceptibility. Cell Rep. 2018;22:1473-1483 pubmed publisher
|
| Pansarasa O, Bordoni M, Drufuca L, Diamanti L, Sproviero D, Trotti R, et al. Lymphoblastoid cell lines as a model to understand amyotrophic lateral sclerosis disease mechanisms. Dis Model Mech. 2018;11: pubmed publisher
|
| Codron P, Cassereau J, Vourc H P, Veyrat Durebex C, Blasco H, Kane S, et al. Primary fibroblasts derived from sporadic amyotrophic lateral sclerosis patients do not show ALS cytological lesions. Amyotroph Lateral Scler Frontotemporal Degener. 2018;19:446-456 pubmed publisher
|
| Pintus R, Riggi M, Cannarozzo C, Valeri A, de Leo G, Romano M, et al. Essential role of hippocampal noradrenaline in the regulation of spatial working memory and TDP-43 tissue pathology. J Comp Neurol. 2018;526:1131-1147 pubmed publisher
|
| Espejo Porras F, Fernandez Ruiz J, de Lago E. Analysis of endocannabinoid receptors and enzymes in the post-mortem motor cortex and spinal cord of amyotrophic lateral sclerosis patients. Amyotroph Lateral Scler Frontotemporal Degener. 2018;19:377-386 pubmed publisher
|
| Gu J, Wang W, Miao S, Chen F, Wu F, Hu W, et al. Protein Phosphatase 1 dephosphorylates TDP-43 and suppresses its function in tau exon 10 inclusion. FEBS Lett. 2018;592:402-410 pubmed publisher
|
| Mishima T, Deshimaru M, Watanabe T, Kubota K, Kinoshita Kawada M, Yuasa Kawada J, et al. Behavioral defects in a DCTN1G71A transgenic mouse model of Perry syndrome. Neurosci Lett. 2018;666:98-103 pubmed publisher
|
| Ayaki T, Ito H, Komure O, Kamada M, Nakamura M, Wate R, et al. Multiple Proteinopathies in Familial ALS Cases With Optineurin Mutations. J Neuropathol Exp Neurol. 2018;77:128-138 pubmed publisher
|
| Chen K, Bennett S, Rana N, Yousuf H, Said M, Taaseen S, et al. Neurodegenerative Disease Proteinopathies Are Connected to Distinct Histone Post-translational Modification Landscapes. ACS Chem Neurosci. 2018;9:838-848 pubmed publisher
|
| So E, Mitchell J, Memmi C, Chennell G, Vizcay Barrena G, Allison L, et al. Mitochondrial abnormalities and disruption of the neuromuscular junction precede the clinical phenotype and motor neuron loss in hFUSWT transgenic mice. Hum Mol Genet. 2018;27:463-474 pubmed publisher
|
| Umoh M, Dammer E, Dai J, Duong D, Lah J, Levey A, et al. A proteomic network approach across the ALS-FTD disease spectrum resolves clinical phenotypes and genetic vulnerability in human brain. EMBO Mol Med. 2018;10:48-62 pubmed publisher
|
| Braak H, Del Tredici K. Anterior Cingulate Cortex TDP-43 Pathology in Sporadic Amyotrophic Lateral Sclerosis. J Neuropathol Exp Neurol. 2018;77:74-83 pubmed publisher
|
| Watanabe R, Kawakami I, Onaya M, Higashi S, Arai N, Akiyama H, et al. Frontotemporal dementia with trans-activation response DNA-binding protein 43 presenting with catatonic syndrome. Neuropathology. 2018;38:281-287 pubmed publisher
|
| Maurel C, Madji Hounoum B, Thepault R, Marouillat S, Brulard C, Danel Brunaud V, et al. Mutation in the RRM2 domain of TDP-43 in Amyotrophic Lateral Sclerosis with rapid progression associated with ubiquitin positive aggregates in cultured motor neurons. Amyotroph Lateral Scler Frontotemporal Degener. 2018;19:149-151 pubmed publisher
|
| Saykally J, Ratliff W, Keeley K, Pick C, Mervis R, Citron B. Repetitive Mild Closed Head Injury Alters Protein Expression and Dendritic Complexity in a Mouse Model. J Neurotrauma. 2018;35:139-148 pubmed publisher
|
| Li S, Chen Y, Sun D, Bai R, Gao X, Yang Y, et al. Angiogenin Prevents Progranulin A9D Mutation-Induced Neuronal-Like Cell Apoptosis Through Cleaving tRNAs into tiRNAs. Mol Neurobiol. 2018;55:1338-1351 pubmed publisher
|
| Redaelli V, Rossi G, Maderna E, Kovacs G, Piccoli E, Caroppo P, et al. Alzheimer neuropathology without frontotemporal lobar degeneration hallmarks (TAR DNA-binding protein 43 inclusions) in missense progranulin mutation Cys139Arg. Brain Pathol. 2018;28:72-76 pubmed publisher
|
| Shmueli M, Hizkiahou N, Peled S, Gazit E, Segal D. Total proteome turbidity assay for tracking global protein aggregation in the natural cellular environment. J Biol Methods. 2017;4:e69 pubmed publisher
|
| Tian K, Jiang H, Wang B, Zhang F, Han S. Intravenous injection of l-BMAA induces a rat model with comprehensive characteristics of amyotrophic lateral sclerosis/Parkinson-dementia complex. Toxicol Res (Camb). 2016;5:79-96 pubmed publisher
|
| Matioli M, Suemoto C, Rodriguez R, Farias D, da Silva M, Leite R, et al. Association between diabetes and causes of dementia: Evidence from a clinicopathological study. Dement Neuropsychol. 2017;11:406-412 pubmed publisher
|
| Liu G, Coyne A, Pei F, Vaughan S, Chaung M, Zarnescu D, et al. Endocytosis regulates TDP-43 toxicity and turnover. Nat Commun. 2017;8:2092 pubmed publisher
|
| Hirsch Reinshagen V, Pottier C, Nicholson A, Baker M, Hsiung G, Krieger C, et al. Clinical and neuropathological features of ALS/FTD with TIA1 mutations. Acta Neuropathol Commun. 2017;5:96 pubmed publisher
|
| Skrott Z, Mistrik M, Andersen K, Friis S, Majera D, Gursky J, et al. Alcohol-abuse drug disulfiram targets cancer via p97 segregase adaptor NPL4. Nature. 2017;552:194-199 pubmed publisher
|
| Mitsuhashi K, Ito D, Mashima K, Oyama M, Takahashi S, Suzuki N. De novo design of RNA-binding proteins with a prion-like domain related to ALS/FTD proteinopathies. Sci Rep. 2017;7:16871 pubmed publisher
|
| Kim S, Stiles S, Feichtmeier J, Ramesh N, Zhan L, Scalf M, et al. Mutation-dependent aggregation and toxicity in a Drosophila model for UBQLN2-associated ALS. Hum Mol Genet. 2017;: pubmed publisher
|
| Huang C, Lee Y, Li P, Liliang P, Lu K, Wang K, et al. TDP-43 proteolysis is associated with astrocyte reactivity after traumatic brain injury in rodents. J Neuroimmunol. 2017;313:61-68 pubmed publisher
|
| Berson A, Sartoris A, Nativio R, Van Deerlin V, Toledo J, Porta S, et al. TDP-43 Promotes Neurodegeneration by Impairing Chromatin Remodeling. Curr Biol. 2017;27:3579-3590.e6 pubmed publisher
|
| Wilhite R, Sage J, Bouzid A, Primavera T, Agbas A. Platelet phosphorylated TDP-43: an exploratory study for a peripheral surrogate biomarker development for Alzheimer's disease. Future Sci OA. 2017;3:FSO238 pubmed publisher
|
| Mosegaard S, Bruun G, Flyvbjerg K, Bliksrud Y, Gregersen N, Dembic M, et al. An intronic variation in SLC52A1 causes exon skipping and transient riboflavin-responsive multiple acyl-CoA dehydrogenation deficiency. Mol Genet Metab. 2017;122:182-188 pubmed publisher
|
| Gregory J, Whiten D, Brown R, Barros T, Kumita J, Yerbury J, et al. Clusterin protects neurons against intracellular proteotoxicity. Acta Neuropathol Commun. 2017;5:81 pubmed publisher
|
| Tsuiji H, Inoue I, Takeuchi M, Furuya A, Yamakage Y, Watanabe S, et al. TDP-43 accelerates age-dependent degeneration of interneurons. Sci Rep. 2017;7:14972 pubmed publisher
|
| Bargsted L, Medinas D, Martínez Traub F, Rozas P, Muñoz N, Nassif M, et al. Disulfide cross-linked multimers of TDP-43 and spinal motoneuron loss in a TDP-43A315T ALS/FTD mouse model. Sci Rep. 2017;7:14266 pubmed publisher
|
| Lyons S, Gudanis D, Coyne S, Gdaniec Z, Ivanov P. Identification of functional tetramolecular RNA G-quadruplexes derived from transfer RNAs. Nat Commun. 2017;8:1127 pubmed publisher
|
| Albayram O, Kondo A, Mannix R, Smith C, Tsai C, Li C, et al. Cis P-tau is induced in clinical and preclinical brain injury and contributes to post-injury sequelae. Nat Commun. 2017;8:1000 pubmed publisher
|
| Dos Santos Matioli M, Suemoto C, Rodriguez R, Farias D, da Silva M, Leite R, et al. Diabetes is Not Associated with Alzheimer's Disease Neuropathology. J Alzheimers Dis. 2017;60:1035-1043 pubmed publisher
|
| Schonhaut D, McMillan C, Spina S, Dickerson B, Siderowf A, Devous M, et al. 18 F-flortaucipir tau positron emission tomography distinguishes established progressive supranuclear palsy from controls and Parkinson disease: A multicenter study. Ann Neurol. 2017;82:622-634 pubmed publisher
|
| Banerjee A, Vest K, Pavlath G, Corbett A. Nuclear poly(A) binding protein 1 (PABPN1) and Matrin3 interact in muscle cells and regulate RNA processing. Nucleic Acids Res. 2017;45:10706-10725 pubmed publisher
|
| Tibshirani M, Zhao B, Gentil B, Minotti S, Marques C, Keith J, et al. Dysregulation of chromatin remodelling complexes in amyotrophic lateral sclerosis. Hum Mol Genet. 2017;26:4142-4152 pubmed publisher
|
| Hawley Z, Campos Melo D, Strong M. Novel miR-b2122 regulates several ALS-related RNA-binding proteins. Mol Brain. 2017;10:46 pubmed publisher
|
| Riku Y, Watanabe H, Yoshida M, Mimuro M, Iwasaki Y, Masuda M, et al. Pathologic Involvement of Glutamatergic Striatal Inputs From the Cortices in TAR DNA-Binding Protein 43?kDa-Related Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis. J Neuropathol Exp Neurol. 2017;76:759-768 pubmed publisher
|
| Takeda T, Seilhean D, Le Ber I, Millecamps S, Sazdovitch V, Kitagawa K, et al. Amygdala TDP-43 Pathology in Frontotemporal Lobar Degeneration and Motor Neuron Disease. J Neuropathol Exp Neurol. 2017;76:800-812 pubmed publisher
|
| Pons M, Miguel L, Miel C, Avequin T, Juge F, Frebourg T, et al. Splicing factors act as genetic modulators of TDP-43 production in a new autoregulatory TDP-43 Drosophila model. Hum Mol Genet. 2017;26:3396-3408 pubmed publisher
|
| Kotaki R, Higuchi H, Ogiya D, Katahira Y, Kurosaki N, Yukihira N, et al. Imbalanced expression of polycistronic miRNA in acute myeloid leukemia. Int J Hematol. 2017;106:811-819 pubmed publisher
|
| Sugiyama K, Aida T, Nomura M, Takayanagi R, Zeilhofer H, Tanaka K. Calpain-Dependent Degradation of Nucleoporins Contributes to Motor Neuron Death in a Mouse Model of Chronic Excitotoxicity. J Neurosci. 2017;37:8830-8844 pubmed publisher
|
| Mackenzie I, Nicholson A, Sarkar M, Messing J, Purice M, Pottier C, et al. TIA1 Mutations in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Promote Phase Separation and Alter Stress Granule Dynamics. Neuron. 2017;95:808-816.e9 pubmed publisher
|
| Mishima T, Koga S, Lin W, Kasanuki K, Castanedes Casey M, Wszolek Z, et al. Perry Syndrome: A Distinctive Type of TDP-43 Proteinopathy. J Neuropathol Exp Neurol. 2017;76:676-682 pubmed publisher
|
| Borroni B, Stanic J, Verpelli C, Mellone M, Bonomi E, Alberici A, et al. Anti-AMPA GluA3 antibodies in Frontotemporal dementia: a new molecular target. Sci Rep. 2017;7:6723 pubmed publisher
|
| Wang P, Wander C, Yuan C, Bereman M, Cohen T. Acetylation-induced TDP-43 pathology is suppressed by an HSF1-dependent chaperone program. Nat Commun. 2017;8:82 pubmed publisher
|
| Paolicelli R, Jawaid A, Henstridge C, Valeri A, Merlini M, Robinson J, et al. TDP-43 Depletion in Microglia Promotes Amyloid Clearance but Also Induces Synapse Loss. Neuron. 2017;95:297-308.e6 pubmed publisher
|
| Davidson Y, Robinson A, Flood L, Rollinson S, Benson B, Asi Y, et al. Heterogeneous ribonuclear protein E2 (hnRNP E2) is associated with TDP-43-immunoreactive neurites in Semantic Dementia but not with other TDP-43 pathological subtypes of Frontotemporal Lobar Degeneration. Acta Neuropathol Commun. 2017;5:54 pubmed publisher
|
| Afroz T, Hock E, Ernst P, Foglieni C, Jambeau M, Gilhespy L, et al. Functional and dynamic polymerization of the ALS-linked protein TDP-43 antagonizes its pathologic aggregation. Nat Commun. 2017;8:45 pubmed publisher
|
| Prpar Mihevc S, Pavlin M, Darovic S, Živin M, Podbregar M, Rogelj B, et al. Modelling FUS Mislocalisation in an In Vitro Model of Innervated Human Muscle. J Mol Neurosci. 2017;62:318-328 pubmed publisher
|
| Osuru H, Pramoonjago P, Abhyankar M, Swanson E, Roker L, Cathro H, et al. Immunolocalization of TAR DNA-binding protein of 43?kDa (TDP-43) in mouse seminiferous epithelium. Mol Reprod Dev. 2017;84:675-685 pubmed publisher
|
| Appocher C, Mohagheghi F, Cappelli S, Stuani C, Romano M, Feiguin F, et al. Major hnRNP proteins act as general TDP-43 functional modifiers both in Drosophila and human neuronal cells. Nucleic Acids Res. 2017;45:8026-8045 pubmed publisher
|
| Mompeán M, Romano V, Pantoja Uceda D, Stuani C, Baralle F, Buratti E, et al. Point mutations in the N-terminal domain of transactive response DNA-binding protein 43 kDa (TDP-43) compromise its stability, dimerization, and functions. J Biol Chem. 2017;292:11992-12006 pubmed publisher
|
| Hall C, Yao Z, Choi M, Tyzack G, Serio A, Luisier R, et al. Progressive Motor Neuron Pathology and the Role of Astrocytes in a Human Stem Cell Model of VCP-Related ALS. Cell Rep. 2017;19:1739-1749 pubmed publisher
|
| Cagnin A, Mariotto S, Fiorini M, Gaule M, Bonetto N, Tagliapietra M, et al. Microglial and Neuronal TDP-43 Pathology in Anti-IgLON5-Related Tauopathy. J Alzheimers Dis. 2017;59:13-20 pubmed publisher
|
| Park S, Hong J, Arslan F, Kanneganti V, Patel B, Tietsort A, et al. Overexpression of the essential Sis1 chaperone reduces TDP-43 effects on toxicity and proteolysis. PLoS Genet. 2017;13:e1006805 pubmed publisher
|
| Cykowski M, Powell S, Peterson L, Appel J, Rivera A, Takei H, et al. Clinical Significance of TDP-43 Neuropathology in Amyotrophic Lateral Sclerosis. J Neuropathol Exp Neurol. 2017;76:402-413 pubmed publisher
|
| Wobst H, Delsing L, Brandon N, Moss S. Truncation of the TAR DNA-binding protein 43 is not a prerequisite for cytoplasmic relocalization, and is suppressed by caspase inhibition and by introduction of the A90V sequence variant. PLoS ONE. 2017;12:e0177181 pubmed publisher
|
| Wang X, Chang L, Wang H, Su A, Wu Z. Dcp1a and GW182 Induce Distinct Cellular Aggregates and Have Different Effects on microRNA Pathway. DNA Cell Biol. 2017;36:565-570 pubmed publisher
|
| Ash P, Stanford E, Al Abdulatif A, Ramirez Cardenas A, Ballance H, Boudeau S, et al. Dioxins and related environmental contaminants increase TDP-43 levels. Mol Neurodegener. 2017;12:35 pubmed publisher
|
| Sheng L, Cai L, Liu J, Zhang S, Xu J, Zhang X, et al. Imaging specific newly synthesized proteins within cells by fluorescence resonance energy transfer. Chem Sci. 2017;8:748-754 pubmed publisher
|
| Lojk J, Prpar Mihevc S, Bregar V, Pavlin M, Rogelj B. The Effect of Different Types of Nanoparticles on FUS and TDP-43 Solubility and Subcellular Localization. Neurotox Res. 2017;32:325-339 pubmed publisher
|
| Davidson Y, Flood L, Robinson A, Nihei Y, Mori K, Rollinson S, et al. Heterogeneous ribonuclear protein A3 (hnRNP A3) is present in dipeptide repeat protein containing inclusions in Frontotemporal Lobar Degeneration and Motor Neurone disease associated with expansions in C9orf72 gene. Acta Neuropathol Commun. 2017;5:31 pubmed publisher
|
| Filareti M, Luotti S, Pasetto L, Pignataro M, Paolella K, Messina P, et al. Decreased Levels of Foldase and Chaperone Proteins Are Associated with an Early-Onset Amyotrophic Lateral Sclerosis. Front Mol Neurosci. 2017;10:99 pubmed publisher
|
| Davis S, Gan K, Dowell J, Cairns N, Gitcho M. TDP-43 expression influences amyloid? plaque deposition and tau aggregation. Neurobiol Dis. 2017;103:154-162 pubmed publisher
|
| Chiu W, Donker Kaat L, Boon A, Kamphorst W, Schleicher A, Zilles K, et al. Multireceptor fingerprints in progressive supranuclear palsy. Alzheimers Res Ther. 2017;9:28 pubmed publisher
|
| Chitramuthu B, Kay D, Bateman A, Bennett H. Neurotrophic effects of progranulin in vivo in reversing motor neuron defects caused by over or under expression of TDP-43 or FUS. PLoS ONE. 2017;12:e0174784 pubmed publisher
|
| Suemoto C, Ferretti Rebustini R, Rodriguez R, Leite R, Soterio L, Brucki S, et al. Neuropathological diagnoses and clinical correlates in older adults in Brazil: A cross-sectional study. PLoS Med. 2017;14:e1002267 pubmed publisher
|
| Goto K, Imamura K, Komatsu K, Mitani K, Aiba K, Nakatsuji N, et al. Simple Derivation of Spinal Motor Neurons from ESCs/iPSCs Using Sendai Virus Vectors. Mol Ther Methods Clin Dev. 2017;4:115-125 pubmed publisher
|
| Ishiguro T, Sato N, Ueyama M, Fujikake N, Sellier C, Kanegami A, et al. Regulatory Role of RNA Chaperone TDP-43 for RNA Misfolding and Repeat-Associated Translation in SCA31. Neuron. 2017;94:108-124.e7 pubmed publisher
|
| Sun M, Bell W, Laclair K, Ling J, Han H, Kageyama Y, et al. Cryptic exon incorporation occurs in Alzheimer's brain lacking TDP-43 inclusion but exhibiting nuclear clearance of TDP-43. Acta Neuropathol. 2017;133:923-931 pubmed publisher
|
| Khalil B, Cabirol Pol M, Miguel L, Whitworth A, Lecourtois M, Liévens J. Enhancing Mitofusin/Marf ameliorates neuromuscular dysfunction in Drosophila models of TDP-43 proteinopathies. Neurobiol Aging. 2017;54:71-83 pubmed publisher
|
| Zhang T, Mishra P, Hay B, Chan D, Guo M. Valosin-containing protein (VCP/p97) inhibitors relieve Mitofusin-dependent mitochondrial defects due to VCP disease mutants. elife. 2017;6: pubmed publisher
|
| Gopal P, Nirschl J, Klinman E, Holzbaur E. Amyotrophic lateral sclerosis-linked mutations increase the viscosity of liquid-like TDP-43 RNP granules in neurons. Proc Natl Acad Sci U S A. 2017;114:E2466-E2475 pubmed publisher
|
| Da Cruz S, Bui A, Saberi S, Lee S, Stauffer J, McAlonis Downes M, et al. Misfolded SOD1 is not a primary component of sporadic ALS. Acta Neuropathol. 2017;134:97-111 pubmed publisher
|
| Li W, Reeb A, Lin B, Subramanian P, Fey E, Knoverek C, et al. Heat Shock-induced Phosphorylation of TAR DNA-binding Protein 43 (TDP-43) by MAPK/ERK Kinase Regulates TDP-43 Function. J Biol Chem. 2017;292:5089-5100 pubmed publisher
|
| Shantanu S, Vijayalakshmi K, Shruthi S, Sagar B, Sathyaprabha T, Nalini A, et al. VEGF alleviates ALS-CSF induced cytoplasmic accumulations of TDP-43 and FUS/TLS in NSC-34 cells. J Chem Neuroanat. 2017;81:48-52 pubmed publisher
|
| Russo A, Scardigli R, La Regina F, Murray M, Romano N, Dickson D, et al. Increased cytoplasmic TDP-43 reduces global protein synthesis by interacting with RACK1 on polyribosomes. Hum Mol Genet. 2017;26:1407-1418 pubmed publisher
|
| Stopford M, Higginbottom A, Hautbergue G, Cooper Knock J, Mulcahy P, De Vos K, et al. C9ORF72 hexanucleotide repeat exerts toxicity in a stable, inducible motor neuronal cell model, which is rescued by partial depletion of Pten. Hum Mol Genet. 2017;26:1133-1145 pubmed publisher
|
| Jeong Y, Ling J, Lin S, Donde A, Braunstein K, Majounie E, et al. Tdp-43 cryptic exons are highly variable between cell types. Mol Neurodegener. 2017;12:13 pubmed publisher
|
| Lee E, Porta S, Michael Baer G, Xu Y, Suh E, Kwong L, et al. Expansion of the classification of FTLD-TDP: distinct pathology associated with rapidly progressive frontotemporal degeneration. Acta Neuropathol. 2017;134:65-78 pubmed publisher
|
| Wang W, Arakawa H, Wang L, Okolo O, Siedlak S, Jiang Y, et al. Motor-Coordinative and Cognitive Dysfunction Caused by Mutant TDP-43 Could Be Reversed by Inhibiting Its Mitochondrial Localization. Mol Ther. 2017;25:127-139 pubmed publisher
|
| Williams S, Khan G, Harris B, Ravits J, Sierks M. TDP-43 protein variants as biomarkers in amyotrophic lateral sclerosis. BMC Neurosci. 2017;18:20 pubmed publisher
|
| Cho K, Yoon D, Qiu S, Danziger Z, Grill W, Wetsel W, et al. Loss of Ranbp2 in motoneurons causes disruption of nucleocytoplasmic and chemokine signaling, proteostasis of hnRNPH3 and Mmp28, and development of amyotrophic lateral sclerosis-like syndromes. Dis Model Mech. 2017;10:559-579 pubmed publisher
|
| Yoshida K, Hata Y, Kinoshita K, Takashima S, Tanaka K, Nishida N. Incipient progressive supranuclear palsy is more common than expected and may comprise clinicopathological subtypes: a forensic autopsy series. Acta Neuropathol. 2017;133:809-823 pubmed publisher
|
| Marquié M, Normandin M, Meltzer A, Siao Tick Chong M, Andrea N, Antón Fernández A, et al. Pathological correlations of [F-18]-AV-1451 imaging in non-alzheimer tauopathies. Ann Neurol. 2017;81:117-128 pubmed publisher
|
| Seo S, Ayakta N, Grinberg L, Villeneuve S, Lehmann M, Reed B, et al. Regional correlations between [11C]PIB PET and post-mortem burden of amyloid-beta pathology in a diverse neuropathological cohort. Neuroimage Clin. 2017;13:130-137 pubmed
|
| Nagahara Y, Shimazawa M, Ohuchi K, Ito J, Takahashi H, Tsuruma K, et al. GPNMB ameliorates mutant TDP-43-induced motor neuron cell death. J Neurosci Res. 2017;95:1647-1665 pubmed publisher
|
| Homma S, Beermann M, Yu B, BOYCE F, Miller J. Nuclear bodies reorganize during myogenesis in vitro and are differentially disrupted by expression of FSHD-associated DUX4. Skelet Muscle. 2016;6:42 pubmed
|
| Wu H, Yin Q, Luo Z, Yao R, Zheng C, Zhang J, et al. Unusual Processing Generates SPA LncRNAs that Sequester Multiple RNA Binding Proteins. Mol Cell. 2016;64:534-548 pubmed publisher
|
| Mufson E, Perez S, Nadeem M, Mahady L, Kanaan N, Abrahamson E, et al. Progression of tau pathology within cholinergic nucleus basalis neurons in chronic traumatic encephalopathy: A chronic effects of neurotrauma consortium study. Brain Inj. 2016;30:1399-1413 pubmed
|
| Altanbyek V, Cha S, Kang G, Im D, Lee S, Kim H, et al. Imbalance of mitochondrial dynamics in Drosophila models of amyotrophic lateral sclerosis. Biochem Biophys Res Commun. 2016;481:259-264 pubmed publisher
|
| Vatsavayai S, Yoon S, Gardner R, Gendron T, Vargas J, Trujillo A, et al. Timing and significance of pathological features in C9orf72 expansion-associated frontotemporal dementia. Brain. 2016;139:3202-3216 pubmed
|
| Braak H, Ludolph A, Neumann M, Ravits J, Del Tredici K. Pathological TDP-43 changes in Betz cells differ from those in bulbar and spinal α-motoneurons in sporadic amyotrophic lateral sclerosis. Acta Neuropathol. 2017;133:79-90 pubmed publisher
|
| Prpar Mihevc S, Baralle M, Buratti E, Rogelj B. TDP-43 aggregation mirrors TDP-43 knockdown, affecting the expression levels of a common set of proteins. Sci Rep. 2016;6:33996 pubmed publisher
|
| Kuiperij H, Versleijen A, Beenes M, Verwey N, Benussi L, Paterlini A, et al. Tau Rather than TDP-43 Proteins are Potential Cerebrospinal Fluid Biomarkers for Frontotemporal Lobar Degeneration Subtypes: A Pilot Study. J Alzheimers Dis. 2017;55:585-595 pubmed
|
| West J, Mito M, Kurosaka S, Takumi T, Tanegashima C, Chujo T, et al. Structural, super-resolution microscopy analysis of paraspeckle nuclear body organization. J Cell Biol. 2016;214:817-30 pubmed publisher
|
| Matsukawa K, Hashimoto T, Matsumoto T, Ihara R, Chihara T, Miura M, et al. Familial Amyotrophic Lateral Sclerosis-linked Mutations in Profilin 1 Exacerbate TDP-43-induced Degeneration in the Retina of Drosophila melanogaster through an Increase in the Cytoplasmic Localization of TDP-43. J Biol Chem. 2016;291:23464-23476 pubmed
|
| Wright D, Liu S, van der Poel C, McDonald S, Brady R, Taylor L, et al. Traumatic Brain Injury Results in Cellular, Structural and Functional Changes Resembling Motor Neuron Disease. Cereb Cortex. 2017;27:4503-4515 pubmed publisher
|
| Armstrong R. Survival in the pre-senile dementia frontotemporal lobar degeneration with TDP-43 proteinopathy: effects of genetic, demographic and neuropathological variables. Folia Neuropathol. 2016;54:137-48 pubmed
|
| Deshpande M, Feiger Z, Shilton A, Luo C, Silverman E, Rodal A. Role of BMP receptor traffic in synaptic growth defects in an ALS model. Mol Biol Cell. 2016;27:2898-910 pubmed publisher
|
| Heyburn L, Hebron M, Smith J, Winston C, Bechara J, Li Z, et al. Tyrosine kinase inhibition reverses TDP-43 effects on synaptic protein expression, astrocytic function and amino acid dis-homeostasis. J Neurochem. 2016;139:610-623 pubmed publisher
|
| Grinberg L, Anghinah R, Nascimento C, Amaro E, Leite R, Martin M, et al. Chronic Traumatic Encephalopathy Presenting as Alzheimer's Disease in a Retired Soccer Player. J Alzheimers Dis. 2016;54:169-74 pubmed publisher
|
| Perera N, Sheean R, Crouch P, White A, Horne M, Turner B. Enhancing survival motor neuron expression extends lifespan and attenuates neurodegeneration in mutant TDP-43 mice. Hum Mol Genet. 2016;25:4080-4093 pubmed publisher
|
| Alves S, Marais T, Biferi M, Furling D, Marinello M, El Hachimi K, et al. Lentiviral vector-mediated overexpression of mutant ataxin-7 recapitulates SCA7 pathology and promotes accumulation of the FUS/TLS and MBNL1 RNA-binding proteins. Mol Neurodegener. 2016;11:58 pubmed publisher
|
| Mori K, Nihei Y, Arzberger T, Zhou Q, Mackenzie I, Hermann A, et al. Reduced hnRNPA3 increases C9orf72 repeat RNA levels and dipeptide-repeat protein deposition. EMBO Rep. 2016;17:1314-25 pubmed publisher
|
| Anderson D, Cannavino J, Li H, Anderson K, Nelson B, McAnally J, et al. Severe muscle wasting and denervation in mice lacking the RNA-binding protein ZFP106. Proc Natl Acad Sci U S A. 2016;113:E4494-503 pubmed publisher
|
| Bruun G, Doktor T, Borch Jensen J, Masuda A, Krainer A, Ohno K, et al. Global identification of hnRNP A1 binding sites for SSO-based splicing modulation. BMC Biol. 2016;14:54 pubmed publisher
|
| Akamatsu M, Yamashita T, Hirose N, Teramoto S, Kwak S. The AMPA receptor antagonist perampanel robustly rescues amyotrophic lateral sclerosis (ALS) pathology in sporadic ALS model mice. Sci Rep. 2016;6:28649 pubmed publisher
|
| Riku Y, Watanabe H, Yoshida M, Mimuro M, Iwasaki Y, Masuda M, et al. Marked Involvement of the Striatal Efferent System in TAR DNA-Binding Protein 43?kDa-Related Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis. J Neuropathol Exp Neurol. 2016;: pubmed
|
| Mishima T, Ishikawa T, Imamura K, Kondo T, Koshiba Y, Takahashi R, et al. Cytoplasmic aggregates of dynactin in iPSC-derived tyrosine hydroxylase-positive neurons from a patient with Perry syndrome. Parkinsonism Relat Disord. 2016;30:67-72 pubmed publisher
|
| Takeuchi R, Tada M, Shiga A, Toyoshima Y, Konno T, Sato T, et al. Heterogeneity of cerebral TDP-43 pathology in sporadic amyotrophic lateral sclerosis: Evidence for clinico-pathologic subtypes. Acta Neuropathol Commun. 2016;4:61 pubmed publisher
|
| Rodriguez R, Suemoto C, Molina M, Nascimento C, Leite R, de Lucena Ferretti Rebustini R, et al. Argyrophilic Grain Disease: Demographics, Clinical, and Neuropathological Features From a Large Autopsy Study. J Neuropathol Exp Neurol. 2016;75:628-35 pubmed publisher
|
| . Defining the spectrum of frontotemporal dementias associated with TARDBP mutations. Neurol Genet. 2016;2:e80 pubmed publisher
|
| Koyama A, Sugai A, Kato T, Ishihara T, Shiga A, Toyoshima Y, et al. Increased cytoplasmic TARDBP mRNA in affected spinal motor neurons in ALS caused by abnormal autoregulation of TDP-43. Nucleic Acids Res. 2016;44:5820-36 pubmed publisher
|
| Dardis A, Zampieri S, Canterini S, Newell K, Stuani C, Murrell J, et al. Altered localization and functionality of TAR DNA Binding Protein 43 (TDP-43) in niemann- pick disease type C. Acta Neuropathol Commun. 2016;4:52 pubmed publisher
|
| Sasaguri H, Chew J, Xu Y, Gendron T, Garrett A, Lee C, et al. The extreme N-terminus of TDP-43 mediates the cytoplasmic aggregation of TDP-43 and associated toxicity in vivo. Brain Res. 2016;1647:57-64 pubmed publisher
|
| Jiang J, Zhu Q, Gendron T, Saberi S, McAlonis Downes M, Seelman A, et al. Gain of Toxicity from ALS/FTD-Linked Repeat Expansions in C9ORF72 Is Alleviated by Antisense Oligonucleotides Targeting GGGGCC-Containing RNAs. Neuron. 2016;90:535-50 pubmed publisher
|
| Oshima R, Hasegawa T, Tamai K, Sugeno N, Yoshida S, Kobayashi J, et al. ESCRT-0 dysfunction compromises autophagic degradation of protein aggregates and facilitates ER stress-mediated neurodegeneration via apoptotic and necroptotic pathways. Sci Rep. 2016;6:24997 pubmed publisher
|
| Langellotti S, Romano V, Romano G, Klima R, Feiguin F, Cragnaz L, et al. A novel Drosophila model of TDP-43 proteinopathies: N-terminal sequences combined with the Q/N domain induce protein functional loss and locomotion defects. Dis Model Mech. 2016;9:659-69 pubmed publisher
|
| Takada L, Bahia V, Guimarães H, Costa T, Vale T, Rodriguez R, et al. GRN and MAPT Mutations in 2 Frontotemporal Dementia Research Centers in Brazil. Alzheimer Dis Assoc Disord. 2016;30:310-317 pubmed
|
| Van den Bossche T, Sleegers K, Cuyvers E, Engelborghs S, Sieben A, De Roeck A, et al. Phenotypic characteristics of Alzheimer patients carrying an ABCA7 mutation. Neurology. 2016;86:2126-33 pubmed publisher
|
| Kedersha N, Panas M, Achorn C, Lyons S, Tisdale S, Hickman T, et al. G3BP-Caprin1-USP10 complexes mediate stress granule condensation and associate with 40S subunits. J Cell Biol. 2016;212:845-60 pubmed publisher
|
| Guo Y, Wang L, Zhu M, Zhang H, Hu Y, Han Z, et al. Detection of hyperphosphorylated tau protein and α-synuclein in spinal cord of patients with Alzheimer's disease. Neuropsychiatr Dis Treat. 2016;12:445-52 pubmed publisher
|
| Jokela M, Huovinen S, Raheem O, Lindfors M, Palmio J, Penttilä S, et al. Distinct Muscle Biopsy Findings in Genetically Defined Adult-Onset Motor Neuron Disorders. PLoS ONE. 2016;11:e0151376 pubmed publisher
|
| Kametani F, Obi T, Shishido T, Akatsu H, Murayama S, Saito Y, et al. Mass spectrometric analysis of accumulated TDP-43 in amyotrophic lateral sclerosis brains. Sci Rep. 2016;6:23281 pubmed publisher
|
| Li Y, Collins M, An J, Geiser R, Tegeler T, Tsantilas K, et al. Immunoprecipitation and mass spectrometry defines an extensive RBM45 protein-protein interaction network. Brain Res. 2016;1647:79-93 pubmed publisher
|
| Cykowski M, Takei H, Van Eldik L, Schmitt F, Jicha G, Powell S, et al. Hippocampal Sclerosis but Not Normal Aging or Alzheimer Disease Is Associated With TDP-43 Pathology in the Basal Forebrain of Aged Persons. J Neuropathol Exp Neurol. 2016;75:397-407 pubmed publisher
|
| Lesage S, Drouet V, Majounie E, Deramecourt V, Jacoupy M, Nicolas A, et al. Loss of VPS13C Function in Autosomal-Recessive Parkinsonism Causes Mitochondrial Dysfunction and Increases PINK1/Parkin-Dependent Mitophagy. Am J Hum Genet. 2016;98:500-513 pubmed publisher
|
| Chen H, Mitchell J, Novoselov S, Miller J, Nishimura A, Scotter E, et al. The heat shock response plays an important role in TDP-43 clearance: evidence for dysfunction in amyotrophic lateral sclerosis. Brain. 2016;139:1417-32 pubmed publisher
|
| Kanekura K, Yagi T, Cammack A, Mahadevan J, Kuroda M, Harms M, et al. Poly-dipeptides encoded by the C9ORF72 repeats block global protein translation. Hum Mol Genet. 2016;25:1803-13 pubmed publisher
|
| Shimonaka S, Nonaka T, Suzuki G, Hisanaga S, Hasegawa M. Templated Aggregation of TAR DNA-binding Protein of 43 kDa (TDP-43) by Seeding with TDP-43 Peptide Fibrils. J Biol Chem. 2016;291:8896-907 pubmed publisher
|
| Ghosh S, Geahlen R. Stress Granules Modulate SYK to Cause Microglial Cell Dysfunction in Alzheimer's Disease. EBioMedicine. 2015;2:1785-98 pubmed publisher
|
| Woehlbier U, Colombo A, Saaranen M, Pérez V, Ojeda J, Bustos F, et al. ALS-linked protein disulfide isomerase variants cause motor dysfunction. EMBO J. 2016;35:845-65 pubmed publisher
|
| Sandell S, Huovinen S, Palmio J, Raheem O, Lindfors M, Zhao F, et al. Diagnostically important muscle pathology in DNAJB6 mutated LGMD1D. Acta Neuropathol Commun. 2016;4:9 pubmed publisher
|
| Hiniker A, Daniels B, Margeta M. T-Cell-Mediated Inflammatory Myopathies in HIV-Positive Individuals: A Histologic Study of 19 Cases. J Neuropathol Exp Neurol. 2016;75:239-45 pubmed publisher
|
| Nonaka T, Suzuki G, Tanaka Y, Kametani F, Hirai S, Okado H, et al. Phosphorylation of TAR DNA-binding Protein of 43 kDa (TDP-43) by Truncated Casein Kinase 1δ Triggers Mislocalization and Accumulation of TDP-43. J Biol Chem. 2016;291:5473-83 pubmed publisher
|
| Uchida T, Tamaki Y, Ayaki T, Shodai A, Kaji S, Morimura T, et al. CUL2-mediated clearance of misfolded TDP-43 is paradoxically affected by VHL in oligodendrocytes in ALS. Sci Rep. 2016;6:19118 pubmed publisher
|
| Yamashita T, Teramoto S, Kwak S. Phosphorylated TDP-43 becomes resistant to cleavage by calpain: A regulatory role for phosphorylation in TDP-43 pathology of ALS/FTLD. Neurosci Res. 2016;107:63-9 pubmed publisher
|
| Ghaoui R, Palmio J, Brewer J, Lek M, Needham M, Evilä A, et al. Mutations in HSPB8 causing a new phenotype of distal myopathy and motor neuropathy. Neurology. 2016;86:391-8 pubmed publisher
|
| Fredericks C, Koestler M, SEELEY W, Miller B, Boxer A, Grinberg L. Primary chronic traumatic encephalopathy in an older patient with late-onset AD phenotype. Neurol Clin Pract. 2015;5:475-479 pubmed
|
| Xia Q, Wang H, Hao Z, Fu C, Hu Q, Gao F, et al. TDP-43 loss of function increases TFEB activity and blocks autophagosome-lysosome fusion. EMBO J. 2016;35:121-42 pubmed publisher
|
| Van Mossevelde S, van der Zee J, Gijselinck I, Engelborghs S, Sieben A, Van Langenhove T, et al. Clinical features of TBK1 carriers compared with C9orf72, GRN and non-mutation carriers in a Belgian cohort. Brain. 2016;139:452-67 pubmed publisher
|
| Hasegawa M, Hara Miyauchi C, Ohta H, Sakimura K, Okano H, Okano H. Analysis of RNA metabolism in peripheral WBCs of TDP-43 KI mice identifies novel biomarkers of ALS. Neurosci Res. 2016;106:12-22 pubmed publisher
|
| Peters O, Cabrera G, Tran H, Gendron T, McKeon J, Metterville J, et al. Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic Mice. Neuron. 2015;88:902-909 pubmed publisher
|
| O Rourke J, Bogdanik L, Muhammad A, Gendron T, Kim K, Austin A, et al. C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD. Neuron. 2015;88:892-901 pubmed publisher
|
| Paudel R, Li A, Hardy J, Bhatia K, Houlden H, Holton J. DYT6 Dystonia: A Neuropathological Study. Neurodegener Dis. 2016;16:273-8 pubmed publisher
|
| Yun S, Woo S, Oh S, Hong S, Choe T, Ye S, et al. Melatonin enhances arsenic trioxide-induced cell death via sustained upregulation of Redd1 expression in breast cancer cells. Mol Cell Endocrinol. 2016;422:64-73 pubmed publisher
|
| Muresan V, Ladescu Muresan Z. Shared Molecular Mechanisms in Alzheimer's Disease and Amyotrophic Lateral Sclerosis: Neurofilament-Dependent Transport of sAPP, FUS, TDP-43 and SOD1, with Endoplasmic Reticulum-Like Tubules. Neurodegener Dis. 2016;16:55-61 pubmed publisher
|
| Feiler M, Strobel B, Freischmidt A, Helferich A, Kappel J, Brewer B, et al. TDP-43 is intercellularly transmitted across axon terminals. J Cell Biol. 2015;211:897-911 pubmed publisher
|
| Zhu J, Cynader M, Jia W. TDP-43 Inhibits NF-κB Activity by Blocking p65 Nuclear Translocation. PLoS ONE. 2015;10:e0142296 pubmed publisher
|
| Davidson Y, Robinson A, Liu X, Wu D, Troakes C, Rollinson S, et al. Neurodegeneration in frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9orf72 is linked to TDP-43 pathology and not associated with aggregated forms of dipeptide repeat proteins. Neuropathol Appl Neurobiol. 2016;42:242-54 pubmed publisher
|
| MacNair L, Xiao S, Miletic D, Ghani M, Julien J, Keith J, et al. MTHFSD and DDX58 are novel RNA-binding proteins abnormally regulated in amyotrophic lateral sclerosis. Brain. 2016;139:86-100 pubmed publisher
|
| Picher Martel V, Dutta K, Phaneuf D, Sobue G, Julien J. Ubiquilin-2 drives NF-κB activity and cytosolic TDP-43 aggregation in neuronal cells. Mol Brain. 2015;8:71 pubmed publisher
|
| Cragnaz L, Klima R, De Conti L, Romano G, Feiguin F, Buratti E, et al. An age-related reduction of brain TBPH/TDP-43 levels precedes the onset of locomotion defects in a Drosophila ALS model. Neuroscience. 2015;311:415-21 pubmed publisher
|
| Colombrita C, Onesto E, Buratti E, de la Grange P, Gumina V, Baralle F, et al. From transcriptomic to protein level changes in TDP-43 and FUS loss-of-function cell models. Biochim Biophys Acta. 2015;1849:1398-410 pubmed publisher
|
| Brettle M, Suchowerska A, Chua S, Ittner L, Fath T. Amyotrophic lateral sclerosis-associated mutant profilin 1 increases dendritic arborisation and spine formation in primary hippocampal neurons. Neurosci Lett. 2015;609:223-8 pubmed publisher
|
| Bevilacqua V, Gioia U, Di Carlo V, Tortorelli A, Colombo T, Bozzoni I, et al. Identification of linc-NeD125, a novel long non coding RNA that hosts miR-125b-1 and negatively controls proliferation of human neuroblastoma cells. RNA Biol. 2015;12:1323-37 pubmed publisher
|
| Tobin W, Popescu B, Lowe V, Pirko I, Parisi J, Kantarci K, et al. Multiple sclerosis masquerading as Alzheimer-type dementia: Clinical, radiological and pathological findings. Mult Scler. 2016;22:698-704 pubmed publisher
|
| Jackson K, Dayton R, Klein R. AAV9 supports wide-scale transduction of the CNS and TDP-43 disease modeling in adult rats. Mol Ther Methods Clin Dev. 2015;2:15036 pubmed publisher
|
| Correia A, Patel P, Dutta K, Julien J. Inflammation Induces TDP-43 Mislocalization and Aggregation. PLoS ONE. 2015;10:e0140248 pubmed publisher
|
| Jackrel M, Yee K, Tariq A, Chen A, Shorter J. Disparate Mutations Confer Therapeutic Gain of Hsp104 Function. ACS Chem Biol. 2015;10:2672-9 pubmed publisher
|
| Ke Y, Van Hummel A, Stevens C, Gladbach A, Ippati S, Bi M, et al. Short-term suppression of A315T mutant human TDP-43 expression improves functional deficits in a novel inducible transgenic mouse model of FTLD-TDP and ALS. Acta Neuropathol. 2015;130:661-78 pubmed publisher
|
| Mackenzie I, Frick P, Grässer F, Gendron T, Petrucelli L, Cashman N, et al. Quantitative analysis and clinico-pathological correlations of different dipeptide repeat protein pathologies in C9ORF72 mutation carriers. Acta Neuropathol. 2015;130:845-61 pubmed publisher
|
| Bengoechea R, Pittman S, Tuck E, True H, Weihl C. Myofibrillar disruption and RNA-binding protein aggregation in a mouse model of limb-girdle muscular dystrophy 1D. Hum Mol Genet. 2015;24:6588-602 pubmed publisher
|
| Marquié M, Normandin M, Vanderburg C, Costantino I, Bien E, Rycyna L, et al. Validating novel tau positron emission tomography tracer [F-18]-AV-1451 (T807) on postmortem brain tissue. Ann Neurol. 2015;78:787-800 pubmed publisher
|
| Emde A, Eitan C, Liou L, Libby R, Rivkin N, Magen I, et al. Dysregulated miRNA biogenesis downstream of cellular stress and ALS-causing mutations: a new mechanism for ALS. EMBO J. 2015;34:2633-51 pubmed publisher
|
| Wear M, Kryndushkin D, O Meally R, Sonnenberg J, Cole R, Shewmaker F. Proteins with Intrinsically Disordered Domains Are Preferentially Recruited to Polyglutamine Aggregates. PLoS ONE. 2015;10:e0136362 pubmed publisher
|
| Kurosawa M, Matsumoto G, Sumikura H, Hatsuta H, Murayama S, Sakurai T, et al. Serine 403-phosphorylated p62/SQSTM1 immunoreactivity in inclusions of neurodegenerative diseases. Neurosci Res. 2016;103:64-70 pubmed publisher
|
| Farrawell N, Lambert Smith I, Warraich S, Blair I, Saunders D, Hatters D, et al. Distinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusions. Sci Rep. 2015;5:13416 pubmed publisher
|
| Ling J, Pletnikova O, Troncoso J, Wong P. TDP-43 repression of nonconserved cryptic exons is compromised in ALS-FTD. Science. 2015;349:650-5 pubmed publisher
|
| Kovacs G, van der Zee J, Hort J, Kristoferitsch W, Leitha T, Höftberger R, et al. Clinicopathological description of two cases with SQSTM1 gene mutation associated with frontotemporal dementia. Neuropathology. 2016;36:27-38 pubmed publisher
|
| Sreedharan J, Neukomm L, Brown R, Freeman M. Age-Dependent TDP-43-Mediated Motor Neuron Degeneration Requires GSK3, hat-trick, and xmas-2. Curr Biol. 2015;25:2130-6 pubmed publisher
|
| Matsumoto A, Suzuki H, Fukatsu R, Shimizu H, Suzuki Y, Hisanaga K. An autopsy case of frontotemporal lobar degeneration with the appearance of fused in sarcoma inclusions (basophilic inclusion body disease) clinically presenting corticobasal syndrome. Neuropathology. 2016;36:77-87 pubmed publisher
|
| Iovino M, Agathou S, González Rueda A, Del Castillo Velasco Herrera M, Borroni B, Alberici A, et al. Early maturation and distinct tau pathology in induced pluripotent stem cell-derived neurons from patients with MAPT mutations. Brain. 2015;138:3345-59 pubmed publisher
|
| Fatima M, Tan R, Halliday G, Kril J. Spread of pathology in amyotrophic lateral sclerosis: assessment of phosphorylated TDP-43 along axonal pathways. Acta Neuropathol Commun. 2015;3:47 pubmed publisher
|
| Bucelli R, Arhzaouy K, Pestronk A, Pittman S, Rojas L, Sue C, et al. SQSTM1 splice site mutation in distal myopathy with rimmed vacuoles. Neurology. 2015;85:665-74 pubmed publisher
|
| Ruggieri A, Brancati F, Zanotti S, Maggi L, Pasanisi M, Saredi S, et al. Complete loss of the DNAJB6 G/F domain and novel missense mutations cause distal-onset DNAJB6 myopathy. Acta Neuropathol Commun. 2015;3:44 pubmed publisher
|
| Ding X, Ma M, Teng J, Teng R, Zhou S, Yin J, et al. Exposure to ALS-FTD-CSF generates TDP-43 aggregates in glioblastoma cells through exosomes and TNTs-like structure. Oncotarget. 2015;6:24178-91 pubmed
|
| Ziskin J, Greicius M, Zhu W, Okumu A, Adams C, Plowey E. Neuropathologic analysis of Tyr69His TTR variant meningovascular amyloidosis with dementia. Acta Neuropathol Commun. 2015;3:43 pubmed publisher
|
| Eggers S, Horn A, Roeber S, Härtig W, Nair G, Reich D, et al. Saccadic Palsy following Cardiac Surgery: Possible Role of Perineuronal Nets. PLoS ONE. 2015;10:e0132075 pubmed publisher
|
| Chou C, Alexeeva O, Yamada S, Pribadi A, Zhang Y, Mo B, et al. PABPN1 suppresses TDP-43 toxicity in ALS disease models. Hum Mol Genet. 2015;24:5154-73 pubmed publisher
|
| Mitchell J, Constable R, So E, Vance C, Scotter E, Glover L, et al. Wild type human TDP-43 potentiates ALS-linked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS. Acta Neuropathol Commun. 2015;3:36 pubmed publisher
|
| Kim K, Lee H, Shim Y, Mook Jung I, Jeon G, Sung J. A phosphomimetic mutant TDP-43 (S409/410E) induces Drosha instability and cytotoxicity in Neuro 2A cells. Biochem Biophys Res Commun. 2015;464:236-43 pubmed publisher
|
| Highley J, Lorente Pons A, Cooper Knock J, Wharton S, Ince P, Shaw P, et al. Motor neurone disease/amyotrophic lateral sclerosis associated with intermediate-length CAG repeat expansions in Ataxin-2 does not have 1C2-positive polyglutamine inclusions. Neuropathol Appl Neurobiol. 2016;42:377-89 pubmed publisher
|
| Korvatska O, Leverenz J, Jayadev S, McMillan P, Kurtz I, Guo X, et al. R47H Variant of TREM2 Associated With Alzheimer Disease in a Large Late-Onset Family: Clinical, Genetic, and Neuropathological Study. JAMA Neurol. 2015;72:920-7 pubmed publisher
|
| Zhang F, Wang W, Siedlak S, Liu Y, Liu J, Jiang K, et al. Miro1 deficiency in amyotrophic lateral sclerosis. Front Aging Neurosci. 2015;7:100 pubmed publisher
|
| Vérièpe J, Fossouo L, Parker J. Neurodegeneration in C. elegans models of ALS requires TIR-1/Sarm1 immune pathway activation in neurons. Nat Commun. 2015;6:7319 pubmed publisher
|
| Koppers M, Blokhuis A, Westeneng H, Terpstra M, Zundel C, Vieira de Sá R, et al. C9orf72 ablation in mice does not cause motor neuron degeneration or motor deficits. Ann Neurol. 2015;78:426-38 pubmed publisher
|
| Yang S, Zhang K, Kariawasam R, Bax M, Fifita J, Ooi L, et al. Evaluation of Skin Fibroblasts from Amyotrophic Lateral Sclerosis Patients for the Rapid Study of Pathological Features. Neurotox Res. 2015;28:138-46 pubmed publisher
|
| Kabuta C, Kono K, Wada K, Kabuta T. 4-Hydroxynonenal induces persistent insolubilization of TDP-43 and alters its intracellular localization. Biochem Biophys Res Commun. 2015;463:82-7 pubmed publisher
|
| Narayan M, Peralta D, Gibson C, Zitnyar A, Jinwal U. An optimized InCell Western screening technique identifies hexachlorophene as a novel potent TDP43 targeting drug. J Biotechnol. 2015;207:34-8 pubmed publisher
|
| Wang H, Lee Y, Huang C, Liliang P, Lu K, Chen H, et al. Traumatic brain injury causes frontotemporal dementia and TDP-43 proteolysis. Neuroscience. 2015;300:94-103 pubmed publisher
|
| Cykowski M, Coon E, Powell S, Jenkins S, Benarroch E, Low P, et al. Expanding the spectrum of neuronal pathology in multiple system atrophy. Brain. 2015;138:2293-309 pubmed publisher
|
| Chew J, Gendron T, Prudencio M, Sasaguri H, Zhang Y, Castanedes Casey M, et al. Neurodegeneration. C9ORF72 repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits. Science. 2015;348:1151-4 pubmed publisher
|
| Cooper Knock J, Higginbottom A, Stopford M, Highley J, Ince P, Wharton S, et al. Antisense RNA foci in the motor neurons of C9ORF72-ALS patients are associated with TDP-43 proteinopathy. Acta Neuropathol. 2015;130:63-75 pubmed publisher
|
| Flanagan E, Duffy J, Whitwell J, Vemuri P, Dickson D, Josephs K. Mixed tau and TDP-43 pathology in a patient with unclassifiable primary progressive aphasia. Neurocase. 2016;22:55-9 pubmed publisher
|
| Kino Y, Washizu C, Kurosawa M, Yamada M, Miyazaki H, Akagi T, et al. FUS/TLS deficiency causes behavioral and pathological abnormalities distinct from amyotrophic lateral sclerosis. Acta Neuropathol Commun. 2015;3:24 pubmed publisher
|
| Muñoz Sáez E, de Munck GarcÃa E, Arahuetes Portero R, MartÃnez A, Solas Alados M, Miguel B. Analysis of β-N-methylamino-L-alanine (L-BMAA) neurotoxicity in rat cerebellum. Neurotoxicology. 2015;48:192-205 pubmed publisher
|
| Ling H, Asi Y, Petrovic I, Ahmed Z, Prashanth L, Hazrati L, et al. Minimal change multiple system atrophy: an aggressive variant?. Mov Disord. 2015;30:960-7 pubmed publisher
|
| Goossens J, Vanmechelen E, Trojanowski J, Lee V, Van Broeckhoven C, van der Zee J, et al. TDP-43 as a possible biomarker for frontotemporal lobar degeneration: a systematic review of existing antibodies. Acta Neuropathol Commun. 2015;3:15 pubmed publisher
|
| Nishida N, Hata Y, Yoshida K, Kinoshita K. Neuropathologic features of suicide victims who presented with acute poststroke depression: significance of association with neurodegenerative disorders. J Neuropathol Exp Neurol. 2015;74:401-10 pubmed publisher
|
| Moreno F, Rabinovici G, Karydas A, Miller Z, Hsu S, Legati A, et al. A novel mutation P112H in the TARDBP gene associated with frontotemporal lobar degeneration without motor neuron disease and abundant neuritic amyloid plaques. Acta Neuropathol Commun. 2015;3:19 pubmed publisher
|
| Aulas A, Caron G, Gkogkas C, Mohamed N, Destroismaisons L, Sonenberg N, et al. G3BP1 promotes stress-induced RNA granule interactions to preserve polyadenylated mRNA. J Cell Biol. 2015;209:73-84 pubmed publisher
|
| Cornejo Olivas M, Torres L, Mata I, Mazzetti P, Rivas D, Cosentino C, et al. A Peruvian family with a novel PARK2 mutation: Clinical and pathological characteristics. Parkinsonism Relat Disord. 2015;21:444-8 pubmed publisher
|
| Freischmidt A, Wieland T, Richter B, Ruf W, Schaeffer V, Müller K, et al. Haploinsufficiency of TBK1 causes familial ALS and fronto-temporal dementia. Nat Neurosci. 2015;18:631-6 pubmed publisher
|
| Rockenstein E, Overk C, Ubhi K, Mante M, Patrick C, Adame A, et al. A novel triple repeat mutant tau transgenic model that mimics aspects of pick's disease and fronto-temporal tauopathies. PLoS ONE. 2015;10:e0121570 pubmed publisher
|
| Finelli M, Liu K, Wu Y, Oliver P, Davies K. Oxr1 improves pathogenic cellular features of ALS-associated FUS and TDP-43 mutations. Hum Mol Genet. 2015;24:3529-44 pubmed publisher
|
| Sabatelli M, Zollino M, Conte A, Del Grande A, Marangi G, Lucchini M, et al. Primary fibroblasts cultures reveal TDP-43 abnormalities in amyotrophic lateral sclerosis patients with and without SOD1 mutations. Neurobiol Aging. 2015;36:2005.e5-2005.e13 pubmed publisher
|
| Rossi S, Serrano A, Gerbino V, Giorgi A, Di Francesco L, Nencini M, et al. Nuclear accumulation of mRNAs underlies G4C2-repeat-induced translational repression in a cellular model of C9orf72 ALS. J Cell Sci. 2015;128:1787-99 pubmed publisher
|
| Xiao S, Sanelli T, Chiang H, Sun Y, Chakrabartty A, Keith J, et al. Low molecular weight species of TDP-43 generated by abnormal splicing form inclusions in amyotrophic lateral sclerosis and result in motor neuron death. Acta Neuropathol. 2015;130:49-61 pubmed publisher
|
| Oyanagi K, Yamazaki M, Hashimoto T, Asakawa M, Wakabayashi K, Takahashi H. Hippocampal sclerosis in the parkinsonism-dementia complex of Guam: quantitative examination of neurons, neurofibrillary tangles, and TDP-43 immunoreactivity in CA1. Neuropathology. 2015;35:224-35 pubmed publisher
|
| Homma S, Beermann M, BOYCE F, Miller J. Expression of FSHD-related DUX4-FL alters proteostasis and induces TDP-43 aggregation. Ann Clin Transl Neurol. 2015;2:151-66 pubmed publisher
|
| Å talekar M, Yin X, Rebolj K, Darovic S, Troakes C, Mayr M, et al. Proteomic analyses reveal that loss of TDP-43 affects RNA processing and intracellular transport. Neuroscience. 2015;293:157-70 pubmed publisher
|
| Williams S, Venkataraman L, Tian H, Khan G, Harris B, Sierks M. Novel atomic force microscopy based biopanning for isolation of morphology specific reagents against TDP-43 variants in amyotrophic lateral sclerosis. J Vis Exp. 2015;: pubmed publisher
|
| Nishida N, Yoshida K, Hata Y, Arai Y, Kinoshita K. Pathological features of preclinical or early clinical stages of corticobasal degeneration: a comparison with advanced cases. Neuropathol Appl Neurobiol. 2015;41:893-905 pubmed publisher
|
| Lauranzano E, Pozzi S, Pasetto L, Stucchi R, Massignan T, Paolella K, et al. Peptidylprolyl isomerase A governs TARDBP function and assembly in heterogeneous nuclear ribonucleoprotein complexes. Brain. 2015;138:974-91 pubmed publisher
|
| Jackson K, Dayton R, Fisher Perkins J, Didier P, Baker K, Weimer M, et al. Initial gene vector dosing for studying symptomatology of amyotrophic lateral sclerosis in non-human primates. J Med Primatol. 2015;44:66-75 pubmed publisher
|
| Li Q, Yokoshi M, Okada H, Kawahara Y. The cleavage pattern of TDP-43 determines its rate of clearance and cytotoxicity. Nat Commun. 2015;6:6183 pubmed publisher
|
| Alquezar C, Esteras N, de la Encarnación A, Moreno F, López de Munain A, MartÃn Requero Ã. Increasing progranulin levels and blockade of the ERK1/2 pathway: upstream and downstream strategies for the treatment of progranulin deficient frontotemporal dementia. Eur Neuropsychopharmacol. 2015;25:386-403 pubmed publisher
|
| Tao Z, Wang H, Xia Q, Li K, Li K, Jiang X, et al. Nucleolar stress and impaired stress granule formation contribute to C9orf72 RAN translation-induced cytotoxicity. Hum Mol Genet. 2015;24:2426-41 pubmed publisher
|
| Steele J, Guella I, Szu Tu C, Lin M, Thompson C, Evans D, et al. Defining neurodegeneration on Guam by targeted genomic sequencing. Ann Neurol. 2015;77:458-68 pubmed publisher
|
| Cohen T, Hwang A, Restrepo C, Yuan C, Trojanowski J, Lee V. An acetylation switch controls TDP-43 function and aggregation propensity. Nat Commun. 2015;6:5845 pubmed publisher
|
| Mutihac R, Alegre Abarrategui J, Gordon D, Farrimond L, Yamasaki Mann M, Talbot K, et al. TARDBP pathogenic mutations increase cytoplasmic translocation of TDP-43 and cause reduction of endoplasmic reticulum Ca²⺠signaling in motor neurons. Neurobiol Dis. 2015;75:64-77 pubmed publisher
|
| Obayashi M, Stevanin G, Synofzik M, Monin M, Duyckaerts C, Sato N, et al. Spinocerebellar ataxia type 36 exists in diverse populations and can be caused by a short hexanucleotide GGCCTG repeat expansion. J Neurol Neurosurg Psychiatry. 2015;86:986-95 pubmed publisher
|
| Liu Y, Atkinson R, Fernandez Martos C, Kirkcaldie M, Cui H, Vickers J, et al. Changes in TDP-43 expression in development, aging, and in the neurofilament light protein knockout mouse. Neurobiol Aging. 2015;36:1151-9 pubmed publisher
|
| Moujalled D, James J, Yang S, Zhang K, Duncan C, Moujalled D, et al. Phosphorylation of hnRNP K by cyclin-dependent kinase 2 controls cytosolic accumulation of TDP-43. Hum Mol Genet. 2015;24:1655-69 pubmed publisher
|
| Yamakawa M, Ito D, Honda T, Kubo K, Noda M, Nakajima K, et al. Characterization of the dipeptide repeat protein in the molecular pathogenesis of c9FTD/ALS. Hum Mol Genet. 2015;24:1630-45 pubmed publisher
|
| Wu Q, Liu M, Huang C, Liu X, Huang B, Li N, et al. Pathogenic Ubqln2 gains toxic properties to induce neuron death. Acta Neuropathol. 2015;129:417-28 pubmed publisher
|
| Suzuki H, Shibagaki Y, Hattori S, Matsuoka M. Nuclear TDP-43 causes neuronal toxicity by escaping from the inhibitory regulation by hnRNPs. Hum Mol Genet. 2015;24:1513-27 pubmed publisher
|
| Aoki N, Murray M, Ogaki K, Fujioka S, Rutherford N, Rademakers R, et al. Hippocampal sclerosis in Lewy body disease is a TDP-43 proteinopathy similar to FTLD-TDP Type A. Acta Neuropathol. 2015;129:53-64 pubmed publisher
|
| Zhan L, Xie Q, Tibbetts R. Opposing roles of p38 and JNK in a Drosophila model of TDP-43 proteinopathy reveal oxidative stress and innate immunity as pathogenic components of neurodegeneration. Hum Mol Genet. 2015;24:757-72 pubmed publisher
|
| Cai M, Lee K, Choi S, Yang E. TDP-43 modification in the hSOD1(G93A) amyotrophic lateral sclerosis mouse model. Neurol Res. 2015;37:253-62 pubmed publisher
|
| Murata H, Hattori T, Maeda H, Takashiba S, Takigawa M, Kido J, et al. Identification of transactivation-responsive DNA-binding protein 43 (TARDBP43; TDP-43) as a novel factor for TNF-α expression upon lipopolysaccharide stimulation in human monocytes. J Periodontal Res. 2015;50:452-60 pubmed publisher
|
| Esteras N, Alquézar C, Bartolomé F, de la Encarnación A, Bermejo Pareja F, Molina J, et al. G1/S Cell Cycle Checkpoint Dysfunction in Lymphoblasts from Sporadic Parkinson's Disease Patients. Mol Neurobiol. 2015;52:386-98 pubmed publisher
|
| Budini M, Romano V, Quadri Z, Buratti E, Baralle F. TDP-43 loss of cellular function through aggregation requires additional structural determinants beyond its C-terminal Q/N prion-like domain. Hum Mol Genet. 2015;24:9-20 pubmed publisher
|
| Kertesz A, Finger E, Murrell J, Chertkow H, Ang L, Baker M, et al. Progressive supranuclear palsy in a family with TDP-43 pathology. Neurocase. 2015;21:178-84 pubmed publisher
|
| Nishimoto Y, Nakagawa S, Hirose T, Okano H, Takao M, Shibata S, et al. The long non-coding RNA nuclear-enriched abundant transcript 1_2 induces paraspeckle formation in the motor neuron during the early phase of amyotrophic lateral sclerosis. Mol Brain. 2013;6:31 pubmed publisher
|
| Dennis J, Citron B. Wobbler mice modeling motor neuron disease display elevated transactive response DNA binding protein. Neuroscience. 2009;158:745-50 pubmed publisher
|