| Published Application/Species/Sample/Dilution | Reference |
|---|
- western blot; mouse; 1:1000; loading ...; fig 5s2a
| Moruno Manchon J, Lejault P, Wang Y, McCauley B, Honarpisheh P, Morales Scheihing D, et al. Small-molecule G-quadruplex stabilizers reveal a novel pathway of autophagy regulation in neurons. elife. 2020;9: pubmed publisher |
- immunohistochemistry - free floating section; mouse; 1:100; loading ...; fig 4d
| Zeitler B, Froelich S, Marlen K, Shivak D, Yu Q, Li D, et al. Allele-selective transcriptional repression of mutant HTT for the treatment of Huntington's disease. Nat Med. 2019;25:1131-1142 pubmed publisher |
- immunohistochemistry - frozen section; mouse; 1:300; loading ...; fig 7a
| Fukuoka M, Takahashi M, Fujita H, Chiyo T, Popiel H, Watanabe S, et al. Supplemental Treatment for Huntington's Disease with miR-132 that Is Deficient in Huntington's Disease Brain. Mol Ther Nucleic Acids. 2018;11:79-90 pubmed publisher |
- immunocytochemistry; human; 1:50; loading ...; fig 4c
| Victor M, Richner M, Olsen H, Lee S, Monteys A, Ma C, et al. Striatal neurons directly converted from Huntington's disease patient fibroblasts recapitulate age-associated disease phenotypes. Nat Neurosci. 2018;21:341-352 pubmed publisher |
- western blot; human; 1:500; loading ...; fig 6
| Kolli N, Lu M, Maiti P, Rossignol J, Dunbar G. CRISPR-Cas9 Mediated Gene-Silencing of the Mutant Huntingtin Gene in an In Vitro Model of Huntington's Disease. Int J Mol Sci. 2017;18: pubmed publisher |
- western blot; human; 1:1000; loading ...; fig 2f
- western blot; brewer's yeast; fig 1c
| Vicente Miranda H, Gomes M, Branco Santos J, Breda C, Lázaro D, Lopes L, et al. Glycation potentiates neurodegeneration in models of Huntington's disease. Sci Rep. 2016;6:36798 pubmed publisher |
- immunohistochemistry - frozen section; mouse; 1:50; loading ...; fig 7a
| Zha J, Liu X, Zhu J, Liu S, Lu S, Xu P, et al. A scFv antibody targeting common oligomeric epitope has potential for treating several amyloidoses. Sci Rep. 2016;6:36631 pubmed publisher |
- western blot; mouse; 1:500; loading ...; fig 8
| Huang Z, Her L. The Ubiquitin Receptor ADRM1 Modulates HAP40-Induced Proteasome Activity. Mol Neurobiol. 2017;54:7382-7400 pubmed publisher |
- western blot; mouse; 1:1000; loading ...; fig 2b
| Guo X, Sun X, Hu D, Wang Y, Fujioka H, Vyas R, et al. VCP recruitment to mitochondria causes mitophagy impairment and neurodegeneration in models of Huntington's disease. Nat Commun. 2016;7:12646 pubmed publisher |
- immunohistochemistry; mouse; 1:100; fig 1
| Agostoni E, Michelazzi S, Maurutto M, Carnemolla A, Ciani Y, Vatta P, et al. Effects of Pin1 Loss in Hdh(Q111) Knock-in Mice. Front Cell Neurosci. 2016;10:110 pubmed publisher |
- immunohistochemistry - frozen section; mouse; 1:500; fig 2
| Bayram Weston Z, Jones L, Dunnett S, Brooks S. Comparison of mHTT Antibodies in Huntington's Disease Mouse Models Reveal Specific Binding Profiles and Steady-State Ubiquitin Levels with Disease Development. PLoS ONE. 2016;11:e0155834 pubmed publisher |
- western blot; mouse; 1:1000; fig 2
| Peng Y, Kim M, Hullinger R, O Riordan K, Burger C, Pehar M, et al. Improved proteostasis in the secretory pathway rescues Alzheimer's disease in the mouse. Brain. 2016;139:937-52 pubmed publisher |
- immunohistochemistry - paraffin section; mouse; 1:50
- western blot; mouse; 1:500
| O Brien R, DeGiacomo F, Holcomb J, Bonner A, Ring K, Zhang N, et al. Integration-independent Transgenic Huntington Disease Fragment Mouse Models Reveal Distinct Phenotypes and Life Span in Vivo. J Biol Chem. 2015;290:19287-306 pubmed publisher |
- immunohistochemistry - frozen section; mouse; fig 6e
| Zhang T, Dong K, Liang W, Xu D, Xia H, Geng J, et al. G-protein-coupled receptors regulate autophagy by ZBTB16-mediated ubiquitination and proteasomal degradation of Atg14L. elife. 2015;4:e06734 pubmed publisher |
- immunohistochemistry - paraffin section; mouse
- immunocytochemistry; mouse; fig 1
| Shen W, Li H, Chen G, Chern Y, Tu P. Mutations in the ubiquitin-binding domain of OPTN/optineurin interfere with autophagy-mediated degradation of misfolded proteins by a dominant-negative mechanism. Autophagy. 2015;11:685-700 pubmed publisher |
- immunohistochemistry - free floating section; mouse; 1:100
| Doria J, de Souza J, Andrade J, Rodrigues H, Guimaraes I, Carvalho T, et al. The mGluR5 positive allosteric modulator, CDPPB, ameliorates pathology and phenotypic signs of a mouse model of Huntington's disease. Neurobiol Dis. 2015;73:163-73 pubmed publisher |
- immunohistochemistry - paraffin section; mouse; 1:500
| Blum D, Herrera F, Francelle L, Mendes T, Basquin M, Obriot H, et al. Mutant huntingtin alters Tau phosphorylation and subcellular distribution. Hum Mol Genet. 2015;24:76-85 pubmed publisher |
- immunohistochemistry - free floating section; human
| Corrochano S, Renna M, Osborne G, Carter S, Stewart M, May J, et al. Reducing Igf-1r levels leads to paradoxical and sexually dimorphic effects in HD mice. PLoS ONE. 2014;9:e105595 pubmed publisher |
- immunocytochemistry; rat; 1:1000
- western blot; rat; 1:1000
| Stansfield K, Bichell T, Bowman A, Guilarte T. BDNF and Huntingtin protein modifications by manganese: implications for striatal medium spiny neuron pathology in manganese neurotoxicity. J Neurochem. 2014;131:655-66 pubmed publisher |
- western blot; mouse; 1:1000
| Zhang H, Petit G, Gaughwin P, Hansen C, Ranganathan S, Zuo X, et al. NGF rescues hippocampal cholinergic neuronal markers, restores neurogenesis, and improves the spatial working memory in a mouse model of Huntington's Disease. J Huntingtons Dis. 2013;2:69-82 pubmed publisher |
- immunohistochemistry; mouse; 1:100
- immunohistochemistry - paraffin section; human; 1:100
| Fernández Nogales M, Cabrera J, Santos Galindo M, Hoozemans J, Ferrer I, Rozemuller A, et al. Huntington's disease is a four-repeat tauopathy with tau nuclear rods. Nat Med. 2014;20:881-5 pubmed publisher |
- immunohistochemistry; human; 1:2000
| Cicchetti F, Lacroix S, Cisbani G, Vallières N, Saint Pierre M, St Amour I, et al. Mutant huntingtin is present in neuronal grafts in Huntington disease patients. Ann Neurol. 2014;76:31-42 pubmed publisher |
- western blot; rat; 1:1000
| Lai A, Lan C, Hasan S, Brown M, McLaurin J. scyllo-Inositol promotes robust mutant Huntingtin protein degradation. J Biol Chem. 2014;289:3666-76 pubmed publisher |
- western blot; mouse; 1:100
| Perucho J, Casarejos M, Gomez A, Ruiz C, Fernandez Estevez M, Muñoz M, et al. Striatal infusion of glial conditioned medium diminishes huntingtin pathology in r6/1 mice. PLoS ONE. 2013;8:e73120 pubmed publisher |
- immunohistochemistry; mouse
| Valor L, Guiretti D, Lopez Atalaya J, Barco A. Genomic landscape of transcriptional and epigenetic dysregulation in early onset polyglutamine disease. J Neurosci. 2013;33:10471-82 pubmed publisher |
- immunocytochemistry; human
| Lu B, Palacino J. A novel human embryonic stem cell-derived Huntington's disease neuronal model exhibits mutant huntingtin (mHTT) aggregates and soluble mHTT-dependent neurodegeneration. FASEB J. 2013;27:1820-9 pubmed publisher |
- immunohistochemistry - frozen section; mouse; 1:250
| Popiel H, Takeuchi T, Fujita H, Yamamoto K, Ito C, Yamane H, et al. Hsp40 gene therapy exerts therapeutic effects on polyglutamine disease mice via a non-cell autonomous mechanism. PLoS ONE. 2012;7:e51069 pubmed publisher |
- immunohistochemistry - free floating section; mouse; 1:300
| Horne E, Coy J, Swinney K, Fung S, Cherry A, Marrs W, et al. Downregulation of cannabinoid receptor 1 from neuropeptide Y interneurons in the basal ganglia of patients with Huntington's disease and mouse models. Eur J Neurosci. 2013;37:429-40 pubmed publisher |
- western blot; mouse; fig 2
| Rahman A, Ekman M, Shakirova Y, Andersson K, Morgelin M, Erjefalt J, et al. Late onset vascular dysfunction in the R6/1 model of Huntington's disease. Eur J Pharmacol. 2013;698:345-53 pubmed publisher |
- immunohistochemistry - free floating section; rat; 1:300
| Yu Taeger L, Petrasch Parwez E, Osmand A, Redensek A, Metzger S, Clemens L, et al. A novel BACHD transgenic rat exhibits characteristic neuropathological features of Huntington disease. J Neurosci. 2012;32:15426-38 pubmed publisher |
| Huang T, Smith R, Bacos K, Song D, Faull R, Waldvogel H, et al. No symphony without bassoon and piccolo: changes in synaptic active zone proteins in Huntington's disease. Acta Neuropathol Commun. 2020;8:77 pubmed publisher |
| Ekman F, Ojala D, Adil M, Lopez P, Schaffer D, Gaj T. CRISPR-Cas9-Mediated Genome Editing Increases Lifespan and Improves Motor Deficits in a Huntington's Disease Mouse Model. Mol Ther Nucleic Acids. 2019;17:829-839 pubmed publisher |
| Ochaba J, Fote G, Kachemov M, Thein S, Yeung S, Lau A, et al. IKKβ slows Huntington's disease progression in R6/1 mice. Proc Natl Acad Sci U S A. 2019;116:10952-10961 pubmed publisher |
| Masnata M, Sciacca G, Maxan A, Bousset L, Denis H, Lauruol F, et al. Demonstration of prion-like properties of mutant huntingtin fibrils in both in vitro and in vivo paradigms. Acta Neuropathol. 2019;137:981-1001 pubmed publisher |
| Zhao Q, Busch B, Jiménez Soto L, Ishikawa Ankerhold H, Massberg S, Terradot L, et al. Integrin but not CEACAM receptors are dispensable for Helicobacter pylori CagA translocation. PLoS Pathog. 2018;14:e1007359 pubmed publisher |
| Maxan A, Mason S, Saint Pierre M, Smith E, Ho A, Harrower T, et al. Outcome of cell suspension allografts in a patient with Huntington's disease. Ann Neurol. 2018;84:950-956 pubmed publisher |
| Novati A, Yu Taeger L, González Menéndez I, Quintanilla Martinez L, Nguyen H. Sexual behavior and testis morphology in the BACHD rat model. PLoS ONE. 2018;13:e0198338 pubmed publisher |
| Brilha S, Wysoczanski R, Whittington A, Friedland J, Porter J. Monocyte Adhesion, Migration, and Extracellular Matrix Breakdown Are Regulated by Integrin ?V?3 in Mycobacterium tuberculosis Infection. J Immunol. 2017;199:982-991 pubmed publisher |
| Ashkenazi A, Bento C, Ricketts T, Vicinanza M, Siddiqi F, Pavel M, et al. Polyglutamine tracts regulate beclin 1-dependent autophagy. Nature. 2017;545:108-111 pubmed publisher |
| Gasset Rosa F, Chillon Marinas C, Goginashvili A, Atwal R, Artates J, Tabet R, et al. Polyglutamine-Expanded Huntingtin Exacerbates Age-Related Disruption of Nuclear Integrity and Nucleocytoplasmic Transport. Neuron. 2017;94:48-57.e4 pubmed publisher |
| Giampietro C, Lionetti M, Costantini G, Mutti F, Zapperi S, La Porta C. Cholesterol impairment contributes to neuroserpin aggregation. Sci Rep. 2017;7:43669 pubmed publisher |
| Benraiss A, Wang S, Herrlinger S, Li X, Chandler Militello D, Mauceri J, et al. Human glia can both induce and rescue aspects of disease phenotype in Huntington disease. Nat Commun. 2016;7:11758 pubmed publisher |
| Naranjo J, Zhang H, Villar D, González P, Dopazo X, Morón Oset J, et al. Activating transcription factor 6 derepression mediates neuroprotection in Huntington disease. J Clin Invest. 2016;126:627-38 pubmed publisher |
| Morozova O, Gupta S, Colby D. Prefibrillar huntingtin oligomers isolated from HD brain potently seed amyloid formation. FEBS Lett. 2015;589:1897-903 pubmed publisher |
| Carty N, Berson N, Tillack K, Thiede C, Scholz D, Kottig K, et al. Characterization of HTT inclusion size, location, and timing in the zQ175 mouse model of Huntington's disease: an in vivo high-content imaging study. PLoS ONE. 2015;10:e0123527 pubmed publisher |
| Cheng H, Chern Y, Chen I, Liu C, Li S, Chun S, et al. Effects on murine behavior and lifespan of selectively decreasing expression of mutant huntingtin allele by supt4h knockdown. PLoS Genet. 2015;11:e1005043 pubmed publisher |
| Berggren K, Chen J, Fox J, Miller J, Dodds L, Dugas B, et al. Neonatal iron supplementation potentiates oxidative stress, energetic dysfunction and neurodegeneration in the R6/2 mouse model of Huntington's disease. Redox Biol. 2015;4:363-74 pubmed publisher |
| Ragauskas S, Leinonen H, Puranen J, Rönkkö S, Nymark S, Gurevicius K, et al. Early retinal function deficit without prominent morphological changes in the R6/2 mouse model of Huntington's disease. PLoS ONE. 2014;9:e113317 pubmed publisher |
| Francelle L, Galvan L, Gaillard M, Guillermier M, Houitte D, Bonvento G, et al. Loss of the thyroid hormone-binding protein Crym renders striatal neurons more vulnerable to mutant huntingtin in Huntington's disease. Hum Mol Genet. 2015;24:1563-73 pubmed publisher |
| Arribat Y, Talmat Amar Y, Paucard A, Lesport P, Bonneaud N, Bauer C, et al. Systemic delivery of P42 peptide: a new weapon to fight Huntington's disease. Acta Neuropathol Commun. 2014;2:86 pubmed publisher |
| Yano H, Baranov S, Baranova O, Kim J, Pan Y, Yablonska S, et al. Inhibition of mitochondrial protein import by mutant huntingtin. Nat Neurosci. 2014;17:822-31 pubmed publisher |
| Yamanaka T, Wong H, Tosaki A, Bauer P, Wada K, Kurosawa M, et al. Large-scale RNA interference screening in mammalian cells identifies novel regulators of mutant huntingtin aggregation. PLoS ONE. 2014;9:e93891 pubmed publisher |
| Proenca C, Stoehr N, Bernhard M, Seger S, Genoud C, Roscic A, et al. Atg4b-dependent autophagic flux alleviates Huntington's disease progression. PLoS ONE. 2013;8:e68357 pubmed publisher |
| Strong M, Southwell A, Yonan J, Hayden M, MacGregor G, Thompson L, et al. Age-Dependent Resistance to Excitotoxicity in Htt CAG140 Mice and the Effect of Strain Background. J Huntingtons Dis. 2012;1:221-41 pubmed publisher |
| Damiano M, Diguet E, Malgorn C, D Aurelio M, Galvan L, Petit F, et al. A role of mitochondrial complex II defects in genetic models of Huntington's disease expressing N-terminal fragments of mutant huntingtin. Hum Mol Genet. 2013;22:3869-82 pubmed publisher |
| Garriga Canut M, Agustín Pavón C, Herrmann F, Sanchez A, Dierssen M, Fillat C, et al. Synthetic zinc finger repressors reduce mutant huntingtin expression in the brain of R6/2 mice. Proc Natl Acad Sci U S A. 2012;109:E3136-45 pubmed publisher |
| Kovalenko M, Dragileva E, St Claire J, Gillis T, Guide J, New J, et al. Msh2 acts in medium-spiny striatal neurons as an enhancer of CAG instability and mutant huntingtin phenotypes in Huntington's disease knock-in mice. PLoS ONE. 2012;7:e44273 pubmed publisher |
| Tada M, Coon E, Osmand A, Kirby P, Martin W, Wieler M, et al. Coexistence of Huntington's disease and amyotrophic lateral sclerosis: a clinicopathologic study. Acta Neuropathol. 2012;124:749-60 pubmed publisher |
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| Schwab C, Yu S, McGeer P. Optineurin is colocalized with ubiquitin in Marinesco bodies. Acta Neuropathol. 2012;123:289-92 pubmed publisher |
| Schwab C, Yu S, McGeer E, McGeer P. Optineurin in Huntington's disease intranuclear inclusions. Neurosci Lett. 2012;506:149-54 pubmed publisher |
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| Reinhart P, Kaltenbach L, Essrich C, Dunn D, Eudailey J, DeMarco C, et al. Identification of anti-inflammatory targets for Huntington's disease using a brain slice-based screening assay. Neurobiol Dis. 2011;43:248-56 pubmed publisher |
| Lin Y, Chen C, Soong B, Wu Y, Chen H, Yeh W, et al. Dysregulated brain creatine kinase is associated with hearing impairment in mouse models of Huntington disease. J Clin Invest. 2011;121:1519-23 pubmed publisher |
| Zadori D, Nyiri G, Szonyi A, Szatmari I, Fulop F, Toldi J, et al. Neuroprotective effects of a novel kynurenic acid analogue in a transgenic mouse model of Huntington's disease. J Neural Transm (Vienna). 2011;118:865-75 pubmed publisher |
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| Giampà C, Laurenti D, Anzilotti S, Bernardi G, Menniti F, Fusco F. Inhibition of the striatal specific phosphodiesterase PDE10A ameliorates striatal and cortical pathology in R6/2 mouse model of Huntington's disease. PLoS ONE. 2010;5:e13417 pubmed publisher |
| Lotz G, Legleiter J, Aron R, Mitchell E, Huang S, Ng C, et al. Hsp70 and Hsp40 functionally interact with soluble mutant huntingtin oligomers in a classic ATP-dependent reaction cycle. J Biol Chem. 2010;285:38183-93 pubmed publisher |
| Kohl Z, Regensburger M, Aigner R, Kandasamy M, Winner B, Aigner L, et al. Impaired adult olfactory bulb neurogenesis in the R6/2 mouse model of Huntington's disease. BMC Neurosci. 2010;11:114 pubmed publisher |
| Klevytska A, Tebbenkamp A, Savonenko A, Borchelt D. Partial depletion of CREB-binding protein reduces life expectancy in a mouse model of Huntington disease. J Neuropathol Exp Neurol. 2010;69:396-404 pubmed publisher |
| Legleiter J, Mitchell E, Lotz G, Sapp E, Ng C, DiFiglia M, et al. Mutant huntingtin fragments form oligomers in a polyglutamine length-dependent manner in vitro and in vivo. J Biol Chem. 2010;285:14777-90 pubmed publisher |
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| Weiss A, Roscic A, Paganetti P. Inducible mutant huntingtin expression in HN10 cells reproduces Huntington's disease-like neuronal dysfunction. Mol Neurodegener. 2009;4:11 pubmed publisher |
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| Illuzzi J, Yerkes S, Parekh Olmedo H, Kmiec E. DNA breakage and induction of DNA damage response proteins precede the appearance of visible mutant huntingtin aggregates. J Neurosci Res. 2009;87:733-47 pubmed publisher |
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| Wang J, Wang C, Orr A, Tydlacka S, Li S, Li X. Impaired ubiquitin-proteasome system activity in the synapses of Huntington's disease mice. J Cell Biol. 2008;180:1177-89 pubmed publisher |
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| Charvin D, Roze E, Perrin V, Deyts C, Betuing S, Pages C, et al. Haloperidol protects striatal neurons from dysfunction induced by mutated huntingtin in vivo. Neurobiol Dis. 2008;29:22-9 pubmed |
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| Ryan A, Zeitlin S, Scrable H. Genetic interaction between expanded murine Hdh alleles and p53 reveal deleterious effects of p53 on Huntington's disease pathogenesis. Neurobiol Dis. 2006;24:419-27 pubmed |
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| Diaz Hernandez M, Torres Peraza J, Salvatori Abarca A, Morán M, Gómez Ramos P, Alberch J, et al. Full motor recovery despite striatal neuron loss and formation of irreversible amyloid-like inclusions in a conditional mouse model of Huntington's disease. J Neurosci. 2005;25:9773-81 pubmed |
| Papalexi E, Persson A, Björkqvist M, Petersen A, Woodman B, Bates G, et al. Reduction of GnRH and infertility in the R6/2 mouse model of Huntington's disease. Eur J Neurosci. 2005;22:1541-6 pubmed |
| Wang Y, Liu W, Wada E, Murata M, Wada K, Kanazawa I. Clinico-pathological rescue of a model mouse of Huntington's disease by siRNA. Neurosci Res. 2005;53:241-9 pubmed |
| Chen Z, Kren B, Wong P, Low W, Steer C. Sleeping Beauty-mediated down-regulation of huntingtin expression by RNA interference. Biochem Biophys Res Commun. 2005;329:646-52 pubmed |
| Takeda Y, Tachibana I, Miyado K, Kobayashi M, Miyazaki T, Funakoshi T, et al. Tetraspanins CD9 and CD81 function to prevent the fusion of mononuclear phagocytes. J Cell Biol. 2003;161:945-56 pubmed |
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