| Published Application/Species/Sample/Dilution | Reference |
|---|
- western blot knockout validation; mouse; 1:4000; loading ...; fig 3f
| Armbruster N, Lattanzi A, Jeavons M, Van Wittenberghe L, Gjata B, Marais T, et al. Efficacy and biodistribution analysis of intracerebroventricular administration of an optimized scAAV9-SMN1 vector in a mouse model of spinal muscular atrophy. Mol Ther Methods Clin Dev. 2016;3:16060 pubmed publisher |
- immunocytochemistry; mouse; 1:300; loading ...
- western blot; mouse; 1:1000; loading ...; fig s2a
| Birsa N, Ule A, Garone M, Tsang B, Mattedi F, Chong P, et al. FUS-ALS mutants alter FMRP phase separation equilibrium and impair protein translation. Sci Adv. 2021;7: pubmed publisher |
- immunohistochemistry; mouse; loading ...; fig 5a
| Van Alstyne M, Tattoli I, Delestrée N, Recinos Y, Workman E, Shihabuddin L, et al. Gain of toxic function by long-term AAV9-mediated SMN overexpression in the sensorimotor circuit. Nat Neurosci. 2021;24:930-940 pubmed publisher |
- immunohistochemistry - paraffin section; mouse; 2.5 ug/ml; loading ...; fig 1d, s2a, 2f
- immunocytochemistry; mouse; 0.8 ug/ml; loading ...; fig 3d
- western blot; mouse; 0.03 ug/ml; loading ...; fig 3a
| Chang W, Xu J, Lin T, Hsu J, Hsieh Li H, Hwu Y, et al. Survival Motor Neuron Protein Participates in Mouse Germ Cell Development and Spermatogonium Maintenance. Int J Mol Sci. 2020;21: pubmed publisher |
- western blot; mouse; 1:20,000; loading ...; fig s17a
| Casci I, Krishnamurthy K, Kour S, Tripathy V, Ramesh N, Anderson E, et al. Muscleblind acts as a modifier of FUS toxicity by modulating stress granule dynamics and SMN localization. Nat Commun. 2019;10:5583 pubmed publisher |
- western blot; human; 1:1000; loading ...; fig 1c
| Das R, Schwintzer L, Vinopal S, Roca E, Sylvester M, Oprişoreanu A, et al. New roles for the de-ubiquitylating enzyme OTUD4 in an RNA-protein network and RNA granules. J Cell Sci. 2019;: pubmed publisher |
- western blot; mouse; 1:4000; loading ...; fig 10a
| Moradi M, Sivadasan R, Saal L, Lüningschrör P, Dombert B, Rathod R, et al. Differential roles of α-, β-, and γ-actin in axon growth and collateral branch formation in motoneurons. J Cell Biol. 2017;216:793-814 pubmed publisher |
- western blot; human; loading ...; fig 1a
| Gribling Burrer A, Leichter M, Wurth L, Huttin A, Schlotter F, Troffer Charlier N, et al. SECIS-binding protein 2 interacts with the SMN complex and the methylosome for selenoprotein mRNP assembly and translation. Nucleic Acids Res. 2017;45:5399-5413 pubmed publisher |
- western blot; mouse; 1:1000; loading ...; fig 7e
- western blot; human; 1:1000; loading ...; fig 1a
- western blot; zebrafish ; 1:1000; loading ...; fig 1d
| Powis R, Karyka E, Boyd P, Côme J, Jones R, Zheng Y, et al. Systemic restoration of UBA1 ameliorates disease in spinal muscular atrophy. JCI Insight. 2016;1:e87908 pubmed publisher |
- immunocytochemistry; human; 1:5000; loading ...; fig 3c
- western blot; mouse; 1:300; loading ...; fig 2c
| Osman E, Washington C, Kaifer K, Mazzasette C, Patitucci T, Florea K, et al. Optimization of Morpholino Antisense Oligonucleotides Targeting the Intronic Repressor Element1 in Spinal Muscular Atrophy. Mol Ther. 2016;24:1592-601 pubmed publisher |
- immunohistochemistry; mouse; 1:1000; loading ...; fig 4c
| Shiihashi G, Ito D, Yagi T, Nihei Y, Ebine T, Suzuki N. Mislocated FUS is sufficient for gain-of-toxic-function amyotrophic lateral sclerosis phenotypes in mice. Brain. 2016;139:2380-94 pubmed publisher |
- western blot; human; fig 1
| Poole A, Hebert M. SMN and coilin negatively regulate dyskerin association with telomerase RNA. Biol Open. 2016;5:726-35 pubmed publisher |
- western blot; mouse; 1:2000; fig 6a
| Seo J, Singh N, Ottesen E, Sivanesan S, Shishimorova M, Singh R. Oxidative Stress Triggers Body-Wide Skipping of Multiple Exons of the Spinal Muscular Atrophy Gene. PLoS ONE. 2016;11:e0154390 pubmed publisher |
- western blot; mouse; 1:2000; fig 3
| Ottesen E, Howell M, Singh N, Seo J, Whitley E, Singh R. Severe impairment of male reproductive organ development in a low SMN expressing mouse model of spinal muscular atrophy. Sci Rep. 2016;6:20193 pubmed publisher |
- western blot; human; loading ...; fig 1a
| So B, Wan L, Zhang Z, Li P, Babiash E, Duan J, et al. A U1 snRNP-specific assembly pathway reveals the SMN complex as a versatile hub for RNP exchange. Nat Struct Mol Biol. 2016;23:225-30 pubmed publisher |
- western blot; human; 1:5000; fig 1
| Sun S, Ling S, Qiu J, Albuquerque C, Zhou Y, Tokunaga S, et al. ALS-causative mutations in FUS/TLS confer gain and loss of function by altered association with SMN and U1-snRNP. Nat Commun. 2015;6:6171 pubmed publisher |
- immunocytochemistry; mouse; 1:2000; fig 5
- western blot; mouse; 1:2000; fig 5
| Lee S, Lee T, Lee E, Kang S, Park A, Kim S, et al. Identification of a subnuclear body involved in sequence-specific cytokine RNA processing. Nat Commun. 2015;6:5791 pubmed publisher |
- immunohistochemistry - paraffin section; human; fig 3
- western blot; human; fig 2
| Tsuiji H, Iguchi Y, Furuya A, Kataoka A, Hatsuta H, Atsuta N, et al. Spliceosome integrity is defective in the motor neuron diseases ALS and SMA. EMBO Mol Med. 2013;5:221-34 pubmed publisher |
- immunocytochemistry; human; loading ...; fig 5
- western blot; human; loading ...; fig 3
- western blot; mouse; loading ...; fig 4
| Carissimi C, Saieva L, Gabanella F, Pellizzoni L. Gemin8 is required for the architecture and function of the survival motor neuron complex. J Biol Chem. 2006;281:37009-16 pubmed |
| Sansa A, Hidalgo I, Miralles M, de la Fuente S, Perez Garcia M, Munell F, et al. Spinal Muscular Atrophy autophagy profile is tissue-dependent: differential regulation between muscle and motoneurons. Acta Neuropathol Commun. 2021;9:122 pubmed publisher |
| Kour S, Rajan D, Fortuna T, Anderson E, Ward C, Lee Y, et al. Loss of function mutations in GEMIN5 cause a neurodevelopmental disorder. Nat Commun. 2021;12:2558 pubmed publisher |
| Burke M, McLaurin D, Logan M, Hebert M. Alteration of 28S rRNA 2'-O-methylation by etoposide correlates with decreased SMN phosphorylation and reduced Drosha levels. Biol Open. 2019;: pubmed publisher |
| Thompson L, Morrison K, Shirran S, Groen E, Gillingwater T, Botting C, et al. Neurochondrin interacts with the SMN protein suggesting a novel mechanism for spinal muscular atrophy pathology. J Cell Sci. 2018;131: pubmed publisher |
| Riessland M, Kaczmarek A, Schneider S, Swoboda K, Löhr H, Bradler C, et al. Neurocalcin Delta Suppression Protects against Spinal Muscular Atrophy in Humans and across Species by Restoring Impaired Endocytosis. Am J Hum Genet. 2017;100:297-315 pubmed publisher |
| Dombert B, Sivadasan R, Simon C, Jablonka S, Sendtner M. Presynaptic localization of Smn and hnRNP R in axon terminals of embryonic and postnatal mouse motoneurons. PLoS ONE. 2014;9:e110846 pubmed publisher |
| Shelkovnikova T, Robinson H, Troakes C, Ninkina N, Buchman V. Compromised paraspeckle formation as a pathogenic factor in FUSopathies. Hum Mol Genet. 2014;23:2298-312 pubmed publisher |
| Briese M, Richter D, Sattelle D, Ulfig N. SMN, the product of the spinal muscular atrophy-determining gene, is expressed widely but selectively in the developing human forebrain. J Comp Neurol. 2006;497:808-16 pubmed |
| Xu H, Somers Z, Robinson M, Hebert M. Tim50a, a nuclear isoform of the mitochondrial Tim50, interacts with proteins involved in snRNP biogenesis. BMC Cell Biol. 2005;6:29 pubmed |
