| Year |
Citation |
Score |
| 2023 |
Reilly A, Yaworski R, Beauvais A, Schneider BL, Kothary R. Long term peripheral AAV9-SMN gene therapy promotes survival in a mouse model of spinal muscular atrophy. Human Molecular Genetics. PMID 38073249 DOI: 10.1093/hmg/ddad202 |
0.308 |
|
| 2022 |
Chehade L, Deguise MO, De Repentigny Y, Yaworski R, Beauvais A, Gagnon S, Hensel N, Kothary R. Suppression of the necroptotic cell death pathways improves survival in mice. Frontiers in Cellular Neuroscience. 16: 972029. PMID 35990890 DOI: 10.3389/fncel.2022.972029 |
0.346 |
|
| 2022 |
Khayrullina G, Alipio-Gloria ZA, Deguise MO, Gagnon S, Chehade L, Stinson M, Belous N, Bergman EM, Lischka FW, Rotty J, Dalgard CL, Kothary R, Johnson KA, Burnett BG. Survival motor neuron protein deficiency alters microglia reactivity. Glia. PMID 35373853 DOI: 10.1002/glia.24177 |
0.317 |
|
| 2020 |
Lynch-Godrei A, De Repentigny Y, Ferrier A, Gagnon S, Kothary R. Dystonin loss-of-function leads to impaired autophagy-endolysosomal pathway dynamics. Biochemistry and Cell Biology = Biochimie Et Biologie Cellulaire. PMID 33347391 DOI: 10.1139/bcb-2020-0557 |
0.308 |
|
| 2020 |
Deguise MO, De Repentigny Y, Tierney A, Beauvais A, Michaud J, Chehade L, Thabet M, Paul B, Reilly A, Gagnon S, Renaud JM, Kothary R. Motor transmission defects with sex differences in a new mouse model of mild spinal muscular atrophy. Ebiomedicine. 55: 102750. PMID 32339936 DOI: 10.1016/J.Ebiom.2020.102750 |
0.328 |
|
| 2020 |
Deguise MO, Beauvais A, Schneider BL, Kothary R. Blood Flow to the Spleen is Altered in a Mouse Model of Spinal Muscular Atrophy. Journal of Neuromuscular Diseases. PMID 32333548 DOI: 10.3233/Jnd-200493 |
0.348 |
|
| 2020 |
Lynch-Godrei A, Kothary R. HSAN-VI: A spectrum disorder based on dystonin isoform expression. Neurology. Genetics. 6: e389. PMID 32042917 DOI: 10.1212/Nxg.0000000000000389 |
0.374 |
|
| 2019 |
Lynch-Godrei A, De Repentigny Y, Yaworski RA, Gagnon S, Butcher J, Manoogian J, Stintzi A, Kothary R. Characterization of gastrointestinal pathologies in the dystonia musculorum mouse model for hereditary sensory and autonomic neuropathy type VI. Neurogastroenterology and Motility : the Official Journal of the European Gastrointestinal Motility Society. e13773. PMID 31814231 DOI: 10.1111/Nmo.13773 |
0.397 |
|
| 2019 |
Alvarez-Saavedra M, Yan K, De Repentigny Y, Hashem LE, Chaudary N, Sarwar S, Yang D, Ioshikhes I, Kothary R, Hirayama T, Yagi T, Picketts DJ. Snf2h Drives Chromatin Remodeling to Prime Upper Layer Cortical Neuron Development. Frontiers in Molecular Neuroscience. 12: 243. PMID 31680852 DOI: 10.3389/Fnmol.2019.00243 |
0.414 |
|
| 2019 |
Deguise MO, Baranello G, Mastella C, Beauvais A, Michaud J, Leone A, De Amicis R, Battezzati A, Dunham C, Selby K, Warman Chardon J, McMillan HJ, Huang YT, Courtney NL, Mole AJ, ... ... Kothary R, et al. Abnormal fatty acid metabolism is a core component of spinal muscular atrophy. Annals of Clinical and Translational Neurology. 6: 1519-1532. PMID 31402618 DOI: 10.1002/Acn3.50855 |
0.355 |
|
| 2019 |
Eshraghi M, Gombar R, De Repentigny Y, Vacratsis PO, Kothary R. Pathologic alterations in the proteome of synaptosomes from a mouse model of spinal muscular atrophy. Journal of Proteome Research. PMID 31262178 DOI: 10.1021/Acs.Jproteome.9B00159 |
0.45 |
|
| 2018 |
Lynch-Godrei A, De Repentigny Y, Gagnon S, Trung MT, Kothary R. Dystonin-A3 upregulation is responsible for maintenance of tubulin acetylation in a less severe dystonia musculorum mouse model for hereditary sensory and autonomic neuropathy type-VI. Human Molecular Genetics. PMID 29982604 DOI: 10.1093/Hmg/Ddy250 |
0.375 |
|
| 2018 |
Walter LM, Deguise MO, Meijboom KE, Betts CA, Ahlskog N, van Westering TLE, Hazell G, McFall E, Kordala A, Hammond SM, Abendroth F, Murray LM, Shorrock HK, Prosdocimo DA, Haldar SM, ... ... Kothary R, et al. Interventions Targeting Glucocorticoid-Krüppel-like Factor 15-Branched-Chain Amino Acid Signaling Improve Disease Phenotypes in Spinal Muscular Atrophy Mice. Ebiomedicine. PMID 29735415 DOI: 10.1016/J.Ebiom.2018.04.024 |
0.353 |
|
| 2017 |
Bowerman M, Murrray LM, Scamps F, Schneider BL, Kothary R, Raoul C. Pathogenic commonalities between spinal muscular atrophy and amyotrophic lateral sclerosis: Converging roads to therapeutic development. European Journal of Medical Genetics. PMID 29313812 DOI: 10.1016/J.Ejmg.2017.12.001 |
0.404 |
|
| 2017 |
Nash LA, McFall ER, Perozzo AM, Turner M, Poulin KL, De Repentigny Y, Burns JK, McMillan HJ, Warman Chardon J, Burger D, Kothary R, Parks RJ. Survival Motor Neuron Protein is Released from Cells in Exosomes: A Potential Biomarker for Spinal Muscular Atrophy. Scientific Reports. 7: 13859. PMID 29066780 DOI: 10.1038/S41598-017-14313-Z |
0.389 |
|
| 2017 |
Deguise MO, Kothary R. New insights into SMA pathogenesis: immune dysfunction and neuroinflammation. Annals of Clinical and Translational Neurology. 4: 522-530. PMID 28695153 DOI: 10.1002/Acn3.423 |
0.328 |
|
| 2017 |
Deguise MO, De Repentigny Y, McFall E, Auclair N, Sad S, Kothary R. Immune dysregulation may contribute to disease pathogenesis in spinal muscular atrophy mice. Human Molecular Genetics. PMID 28108555 DOI: 10.1093/Hmg/Ddw434 |
0.423 |
|
| 2017 |
O'Meara RW, Cummings SE, De Repentigny Y, McFall E, Michalski JP, Deguise MO, Gibeault S, Kothary R. Oligodendrocyte development and CNS myelination are unaffected in a mouse model of severe spinal muscular atrophy. Human Molecular Genetics. PMID 28069797 DOI: 10.1093/Hmg/Ddw385 |
0.439 |
|
| 2016 |
Eshraghi M, McFall E, Gibeault S, Kothary R. Effect of genetic background on the phenotype of the Smn2B/- mouse model of spinal muscular atrophy. Human Molecular Genetics. 25: 4494-4506. PMID 28172892 DOI: 10.1093/Hmg/Ddw278 |
0.415 |
|
| 2016 |
Nash LA, Burns JK, Chardon JW, Kothary R, Parks RJ. Spinal Muscular Atrophy: More than a Disease of Motor Neurons? Current Molecular Medicine. PMID 27894243 DOI: 10.2174/1566524016666161128113338 |
0.429 |
|
| 2016 |
Alvarez-Saavedra M, De Repentigny Y, Yang D, O'Meara RW, Yan K, Hashem LE, Racacho L, Ioshikhes I, Bulman DE, Parks RJ, Kothary R, Picketts DJ. Voluntary Running Triggers VGF-Mediated Oligodendrogenesis to Prolong the Lifespan of Snf2h-Null Ataxic Mice. Cell Reports. 17: 862-875. PMID 27732860 DOI: 10.1016/J.Celrep.2016.09.030 |
0.354 |
|
| 2016 |
Burns JK, Kothary R, Parks RJ. Opening the window: The case for carrier and perinatal screening for spinal muscular atrophy. Neuromuscular Disorders : Nmd. PMID 27460292 DOI: 10.1016/J.Nmd.2016.06.459 |
0.415 |
|
| 2016 |
Deguise MO, Boyer JG, McFall ER, Yazdani A, De Repentigny Y, Kothary R. Differential induction of muscle atrophy pathways in two mouse models of spinal muscular atrophy. Scientific Reports. 6: 28846. PMID 27349908 DOI: 10.1038/Srep28846 |
0.432 |
|
| 2016 |
Kornfeld SF, Lynch-Godrei A, Bonin SR, Gibeault S, De Repentigny Y, Kothary R. Cytoskeletal Linker Protein Dystonin Is Not Critical to Terminal Oligodendrocyte Differentiation or CNS Myelination. Plos One. 11: e0149201. PMID 26886550 DOI: 10.1371/Journal.Pone.0149201 |
0.377 |
|
| 2016 |
O'Meara RW, Cummings SE, Michalski JP, Kothary R. A new in vitro mouse oligodendrocyte precursor cell migration assay reveals a role for integrin-linked kinase in cell motility. Bmc Neuroscience. 17: 7. PMID 26831726 DOI: 10.1186/S12868-016-0242-2 |
0.312 |
|
| 2016 |
Lynch-Godrei A, Kothary R. Functional and Genetic Analysis of Neuronal Isoforms of BPAG1. Methods in Enzymology. 569: 355-72. PMID 26778567 DOI: 10.1016/Bs.Mie.2015.05.004 |
0.413 |
|
| 2015 |
Murray LM, Beauvais A, Gibeault S, Courtney NL, Kothary R. Transcriptional profiling of differentially vulnerable motor neurons at pre-symptomatic stage in the Smn (2b/-) mouse model of spinal muscular atrophy. Acta Neuropathologica Communications. 3: 55. PMID 26374403 DOI: 10.1186/S40478-015-0231-1 |
0.382 |
|
| 2015 |
Ferrier A, De Repentigny Y, Lynch-Godrei A, Gibeault S, Eid W, Kuo D, Zha X, Kothary R. Disruption in the autophagic process underlies the sensory neuropathy in dystonia musculorum mice. Autophagy. 11: 1025-36. PMID 26043942 DOI: 10.1080/15548627.2015.1052207 |
0.454 |
|
| 2014 |
McFall ER, Murray LM, Lunde JA, Jasmin BJ, Kothary R, Parks RJ. A reduction in the human adenovirus virion size through use of a shortened fibre protein does not enhance muscle transduction following systemic or localised delivery in mice. Virology. 468: 444-53. PMID 25243333 DOI: 10.1016/J.Virol.2014.08.026 |
0.352 |
|
| 2014 |
Liu H, Yazdani A, Murray LM, Beauvais A, Kothary R. The Smn-independent beneficial effects of trichostatin A on an intermediate mouse model of spinal muscular atrophy. Plos One. 9: e101225. PMID 24984019 DOI: 10.1371/Journal.Pone.0101225 |
0.434 |
|
| 2014 |
Alvarez-Saavedra M, De Repentigny Y, Lagali PS, Raghu Ram EV, Yan K, Hashem E, Ivanochko D, Huh MS, Yang D, Mears AJ, Todd MA, Corcoran CP, Bassett EA, Tokarew NJ, Kokavec J, ... ... Kothary R, et al. Snf2h-mediated chromatin organization and histone H1 dynamics govern cerebellar morphogenesis and neural maturation. Nature Communications. 5: 4181. PMID 24946904 DOI: 10.1038/Ncomms5181 |
0.347 |
|
| 2014 |
Hettich J, Ryan SD, de Souza ON, Saraiva Macedo Timmers LF, Tsai S, Atai NA, da Hora CC, Zhang X, Kothary R, Snapp E, Ericsson M, Grundmann K, Breakefield XO, Nery FC. Biochemical and cellular analysis of human variants of the DYT1 dystonia protein, TorsinA/TOR1A. Human Mutation. 35: 1101-13. PMID 24930953 DOI: 10.1002/Humu.22602 |
0.588 |
|
| 2014 |
Boyer JG, Deguise MO, Murray LM, Yazdani A, De Repentigny Y, Boudreau-Larivière C, Kothary R. Myogenic program dysregulation is contributory to disease pathogenesis in spinal muscular atrophy. Human Molecular Genetics. 23: 4249-59. PMID 24691550 DOI: 10.1093/Hmg/Ddu142 |
0.454 |
|
| 2014 |
Bowerman M, Michalski JP, Beauvais A, Murray LM, DeRepentigny Y, Kothary R. Defects in pancreatic development and glucose metabolism in SMN-depleted mice independent of canonical spinal muscular atrophy neuromuscular pathology. Human Molecular Genetics. 23: 3432-44. PMID 24497575 DOI: 10.1093/Hmg/Ddu052 |
0.38 |
|
| 2014 |
Murray L, Gillingwater TH, Kothary R. Dissection of the transversus abdominis muscle for whole-mount neuromuscular junction analysis. Journal of Visualized Experiments : Jove. e51162. PMID 24457471 DOI: 10.3791/51162 |
0.365 |
|
| 2014 |
Ferrier A, Sato T, De Repentigny Y, Gibeault S, Bhanot K, O'Meara RW, Lynch-Godrei A, Kornfeld SF, Young KG, Kothary R. Transgenic expression of neuronal dystonin isoform 2 partially rescues the disease phenotype of the dystonia musculorum mouse model of hereditary sensory autonomic neuropathy VI. Human Molecular Genetics. 23: 2694-710. PMID 24381311 DOI: 10.1093/Hmg/Ddt663 |
0.421 |
|
| 2013 |
Boyer JG, Ferrier A, Kothary R. More than a bystander: the contributions of intrinsic skeletal muscle defects in motor neuron diseases. Frontiers in Physiology. 4: 356. PMID 24391590 DOI: 10.3389/Fphys.2013.00356 |
0.411 |
|
| 2013 |
Boyer JG, Murray LM, Scott K, De Repentigny Y, Renaud JM, Kothary R. Early onset muscle weakness and disruption of muscle proteins in mouse models of spinal muscular atrophy. Skeletal Muscle. 3: 24. PMID 24119341 DOI: 10.1186/2044-5040-3-24 |
0.397 |
|
| 2013 |
Goulet BB, McFall ER, Wong CM, Kothary R, Parks RJ. Supraphysiological expression of survival motor neuron protein from an adenovirus vector does not adversely affect cell function. Biochemistry and Cell Biology = Biochimie Et Biologie Cellulaire. 91: 252-64. PMID 23859020 DOI: 10.1139/Bcb-2012-0094 |
0.442 |
|
| 2013 |
Liu Y, Chakroun I, Yang D, Horner E, Liang J, Aziz A, Chu A, De Repentigny Y, Dilworth FJ, Kothary R, Blais A. Six1 regulates MyoD expression in adult muscle progenitor cells. Plos One. 8: e67762. PMID 23840772 DOI: 10.1371/Journal.Pone.0067762 |
0.362 |
|
| 2013 |
Storbeck CJ, Al-Zahrani KN, Sriram R, Kawesa S, O'Reilly P, Daniel K, McKay M, Kothary R, Tsilfidis C, Sabourin LA. Distinct roles for Ste20-like kinase SLK in muscle function and regeneration. Skeletal Muscle. 3: 16. PMID 23815977 DOI: 10.1186/2044-5040-3-16 |
0.346 |
|
| 2013 |
O'Meara RW, Michalski JP, Anderson C, Bhanot K, Rippstein P, Kothary R. Integrin-linked kinase regulates process extension in oligodendrocytes via control of actin cytoskeletal dynamics. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 33: 9781-93. PMID 23739974 DOI: 10.1523/Jneurosci.5582-12.2013 |
0.331 |
|
| 2013 |
Goulet BB, Kothary R, Parks RJ. At the "junction" of spinal muscular atrophy pathogenesis: the role of neuromuscular junction dysfunction in SMA disease progression. Current Molecular Medicine. 13: 1160-74. PMID 23514457 DOI: 10.2174/15665240113139990044 |
0.435 |
|
| 2013 |
Ferrier A, Boyer JG, Kothary R. Cellular and molecular biology of neuronal dystonin. International Review of Cell and Molecular Biology. 300: 85-120. PMID 23273860 DOI: 10.1016/B978-0-12-405210-9.00003-5 |
0.408 |
|
| 2013 |
Sanchez G, Dury AY, Murray LM, Biondi O, Tadesse H, El Fatimy R, Kothary R, Charbonnier F, Khandjian EW, Côté J. A novel function for the survival motoneuron protein as a translational regulator. Human Molecular Genetics. 22: 668-84. PMID 23136128 DOI: 10.1093/Hmg/Dds474 |
0.37 |
|
| 2013 |
Murray LM, Beauvais A, Bhanot K, Kothary R. Defects in neuromuscular junction remodelling in the Smn(2B/-) mouse model of spinal muscular atrophy. Neurobiology of Disease. 49: 57-67. PMID 22960106 DOI: 10.1016/J.Nbd.2012.08.019 |
0.43 |
|
| 2013 |
Alvarez-Saavedra M, Lagali P, Yan K, Hashem E, Mears A, De Repentigny Y, Wallace VA, Kothary R, Stopka T, Skoultchi AI, Picketts DJ. Coordinated epigenetic regulation of Engrailed-1 by the chromatin remodelers Smarca1 and Smarca5 mediates cerebellar morphogenesis Epigenetics & Chromatin. 6. DOI: 10.1186/1756-8935-6-S1-P105 |
0.401 |
|
| 2012 |
Bowerman M, Swoboda KJ, Michalski JP, Wang GS, Reeks C, Beauvais A, Murphy K, Woulfe J, Screaton RA, Scott FW, Kothary R. Glucose metabolism and pancreatic defects in spinal muscular atrophy. Annals of Neurology. 72: 256-68. PMID 22926856 DOI: 10.1002/Ana.23582 |
0.321 |
|
| 2012 |
Ryan SD, Ferrier A, Kothary R. A novel role for the cytoskeletal linker protein dystonin in the maintenance of microtubule stability and the regulation of ER-Golgi transport. Bioarchitecture. 2: 2-5. PMID 22754620 |
0.544 |
|
| 2012 |
Ghanem N, Andrusiak MG, Svoboda D, Al Lafi SM, Julian LM, McClellan KA, De Repentigny Y, Kothary R, Ekker M, Blais A, Park DS, Slack RS. The Rb/E2F pathway modulates neurogenesis through direct regulation of the Dlx1/Dlx2 bigene cluster. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 32: 8219-30. PMID 22699903 DOI: 10.1523/Jneurosci.1344-12.2012 |
0.348 |
|
| 2012 |
Atai NA, Ryan SD, Kothary R, Breakefield XO, Nery FC. Untethering the nuclear envelope and cytoskeleton: biologically distinct dystonias arising from a common cellular dysfunction. International Journal of Cell Biology. 2012: 634214. PMID 22611399 DOI: 10.1155/2012/634214 |
0.639 |
|
| 2012 |
Ryan SD, Bhanot K, Ferrier A, De Repentigny Y, Chu A, Blais A, Kothary R. Microtubule stability, Golgi organization, and transport flux require dystonin-a2-MAP1B interaction. The Journal of Cell Biology. 196: 727-42. PMID 22412020 DOI: 10.1083/Jcb.201107096 |
0.634 |
|
| 2012 |
Bowerman M, Murray LM, Boyer JG, Anderson CL, Kothary R. Fasudil improves survival and promotes skeletal muscle development in a mouse model of spinal muscular atrophy. Bmc Medicine. 10: 24. PMID 22397316 DOI: 10.1186/1741-7015-10-24 |
0.449 |
|
| 2012 |
Ryan SD, Ferrier A, Sato T, O'Meara RW, De Repentigny Y, Jiang SX, Hou ST, Kothary R. Neuronal dystonin isoform 2 is a mediator of endoplasmic reticulum structure and function. Molecular Biology of the Cell. 23: 553-66. PMID 22190742 DOI: 10.1091/Mbc.E11-06-0573 |
0.62 |
|
| 2012 |
Bowerman M, Murray LM, Beauvais A, Pinheiro B, Kothary R. A critical smn threshold in mice dictates onset of an intermediate spinal muscular atrophy phenotype associated with a distinct neuromuscular junction pathology. Neuromuscular Disorders : Nmd. 22: 263-76. PMID 22071333 DOI: 10.1016/J.Nmd.2011.09.007 |
0.453 |
|
| 2012 |
Ghorbani M, Naas T, Soare C, Kothary R, Diaz-Mitoma F. Microarray analysis of gene expression in the liver of transgenic mouse model of HCV infection Advances in Bioscience and Biotechnology. 3: 1151-1159. DOI: 10.4236/Abb.2012.38141 |
0.323 |
|
| 2011 |
Bhanot K, Young KG, Kothary R. MAP1B and clathrin are novel interacting partners of the giant cyto-linker dystonin. Journal of Proteome Research. 10: 5118-27. PMID 21936565 DOI: 10.1021/Pr200564G |
0.348 |
|
| 2011 |
O'Meara RW, Ryan SD, Colognato H, Kothary R. Derivation of enriched oligodendrocyte cultures and oligodendrocyte/neuron myelinating co-cultures from post-natal murine tissues. Journal of Visualized Experiments : Jove. PMID 21876528 DOI: 10.3791/3324 |
0.575 |
|
| 2011 |
De Repentigny Y, Ferrier A, Ryan SD, Sato T, Kothary R. Motor unit abnormalities in Dystonia musculorum mice. Plos One. 6: e21093. PMID 21698255 DOI: 10.1371/Journal.Pone.0021093 |
0.642 |
|
| 2011 |
O'Meara RW, Michalski JP, Kothary R. Integrin signaling in oligodendrocytes and its importance in CNS myelination. Journal of Signal Transduction. 2011: 354091. PMID 21637375 DOI: 10.1155/2011/354091 |
0.303 |
|
| 2011 |
Michalski JP, Anderson C, Beauvais A, De Repentigny Y, Kothary R. The proteolipid protein promoter drives expression outside of the oligodendrocyte lineage during embryonic and early postnatal development. Plos One. 6: e19772. PMID 21572962 DOI: 10.1371/Journal.Pone.0019772 |
0.401 |
|
| 2011 |
Liu H, Beauvais A, Baker AN, Tsilfidis C, Kothary R. Smn deficiency causes neuritogenesis and neurogenesis defects in the retinal neurons of a mouse model of spinal muscular atrophy. Developmental Neurobiology. 71: 153-69. PMID 20862739 DOI: 10.1002/Dneu.20840 |
0.422 |
|
| 2011 |
Ferrier A, Ryan SD, Kothary R, McNally EM. New Directions in Biology and Disease of Skeletal Muscle, Meeting Report, 5–8 May 2010, Ottawa, Canada Neuromuscular Disorders. 21: 157-159. DOI: 10.1016/J.Nmd.2010.08.009 |
0.556 |
|
| 2010 |
Hammond SM, Gogliotti RG, Rao V, Beauvais A, Kothary R, DiDonato CJ. Mouse survival motor neuron alleles that mimic SMN2 splicing and are inducible rescue embryonic lethality early in development but not late. Plos One. 5: e15887. PMID 21249120 DOI: 10.1371/Journal.Pone.0015887 |
0.432 |
|
| 2010 |
Boyer JG, Bhanot K, Kothary R, Boudreau-Larivière C. Hearts of dystonia musculorum mice display normal morphological and histological features but show signs of cardiac stress. Plos One. 5: e9465. PMID 20209123 DOI: 10.1371/Journal.Pone.0009465 |
0.343 |
|
| 2010 |
Shafey D, Boyer JG, Bhanot K, Kothary R. Identification of novel interacting protein partners of SMN using tandem affinity purification. Journal of Proteome Research. 9: 1659-69. PMID 20201562 DOI: 10.1021/Pr9006987 |
0.428 |
|
| 2010 |
Bowerman M, Beauvais A, Anderson CL, Kothary R. Rho-kinase inactivation prolongs survival of an intermediate SMA mouse model. Human Molecular Genetics. 19: 1468-78. PMID 20097679 DOI: 10.1093/Hmg/Ddq021 |
0.427 |
|
| 2010 |
Miura P, Coriati A, Bélanger G, De Repentigny Y, Lee J, Kothary R, Holcik M, Jasmin BJ. The utrophin A 5'-UTR drives cap-independent translation exclusively in skeletal muscles of transgenic mice and interacts with eEF1A2. Human Molecular Genetics. 19: 1211-20. PMID 20053670 DOI: 10.1093/Hmg/Ddp591 |
0.386 |
|
| 2010 |
Liu H, Shafey D, Moores JN, Kothary R. Neurodevelopmental consequences of Smn depletion in a mouse model of spinal muscular atrophy. Journal of Neuroscience Research. 88: 111-22. PMID 19642194 DOI: 10.1002/Jnr.22189 |
0.456 |
|
| 2010 |
Michaud JL, Stitt-Cavanaugh E, Endlich N, Endlich K, De Repentigny Y, Kothary R, Kennedy CR. Mice with podocyte-specific overexpression of wild type alpha-actinin-4 are healthy controls for K256E-alpha-actinin-4 mutant transgenic mice. Transgenic Research. 19: 285-9. PMID 19585264 DOI: 10.1007/S11248-009-9305-9 |
0.36 |
|
| 2010 |
Boyer JG, Bowerman M, Kothary R. The many faces of SMN: Deciphering the function critical to spinal muscular atrophy pathogenesis Future Neurology. 5: 873-890. DOI: 10.2217/Fnl.10.57 |
0.437 |
|
| 2009 |
Savory JG, Bouchard N, Pierre V, Rijli FM, De Repentigny Y, Kothary R, Lohnes D. Cdx2 regulation of posterior development through non-Hox targets. Development (Cambridge, England). 136: 4099-110. PMID 19906845 DOI: 10.1242/Dev.041582 |
0.354 |
|
| 2009 |
Fernando P, Sandoz JS, Ding W, de Repentigny Y, Brunette S, Kelly JF, Kothary R, Megeney LA. Bin1 SRC homology 3 domain acts as a scaffold for myofiber sarcomere assembly. The Journal of Biological Chemistry. 284: 27674-86. PMID 19633357 DOI: 10.1074/Jbc.M109.029538 |
0.351 |
|
| 2009 |
Bowerman M, Anderson CL, Beauvais A, Boyl PP, Witke W, Kothary R. SMN, profilin IIa and plastin 3: a link between the deregulation of actin dynamics and SMA pathogenesis. Molecular and Cellular Neurosciences. 42: 66-74. PMID 19497369 DOI: 10.1016/J.Mcn.2009.05.009 |
0.436 |
|
| 2008 |
Young KG, Kothary R. Dystonin/Bpag1 is a necessary endoplasmic reticulum/nuclear envelope protein in sensory neurons. Experimental Cell Research. 314: 2750-61. PMID 18638474 DOI: 10.1016/J.Yexcr.2008.06.021 |
0.447 |
|
| 2008 |
Ichikawa H, Terayama R, Yamaai T, De Repentigny Y, Kothary R, Sugimoto T. The number of nociceptors in the trigeminal ganglion but not proprioceptors in the mesencephalic trigeminal tract nucleus is reduced in dystonin deficient dystonia musculorum mice. Brain Research. 1226: 33-8. PMID 18619576 DOI: 10.1016/J.Brainres.2008.05.063 |
0.399 |
|
| 2008 |
Mattis VB, Bowerman M, Kothary R, Lorson CL. A SMNDelta7 read-through product confers functionality to the SMNDelta7 protein. Neuroscience Letters. 442: 54-8. PMID 18601974 DOI: 10.1016/J.Neulet.2008.06.059 |
0.39 |
|
| 2008 |
Shafey D, MacKenzie AE, Kothary R. Neurodevelopmental abnormalities in neurosphere-derived neural stem cells from SMN-depleted mice. Journal of Neuroscience Research. 86: 2839-47. PMID 18521935 DOI: 10.1002/Jnr.21743 |
0.42 |
|
| 2007 |
Bowerman M, Shafey D, Kothary R. Smn depletion alters profilin II expression and leads to upregulation of the RhoA/ROCK pathway and defects in neuronal integrity. Journal of Molecular Neuroscience : Mn. 32: 120-31. PMID 17873296 DOI: 10.1007/S12031-007-0024-5 |
0.412 |
|
| 2007 |
Young KG, Kothary R. Dystonin/Bpag1--a link to what? Cell Motility and the Cytoskeleton. 64: 897-905. PMID 17849487 DOI: 10.1002/Cm.20235 |
0.42 |
|
| 2007 |
Ichikawa H, Terayama R, Yamaai T, De Repentigny Y, Kothary R, Sugimoto T. Dystonin deficiency reduces taste buds and fungiform papillae in the anterior part of the tongue. Brain Research. 1129: 142-6. PMID 17156752 DOI: 10.1016/J.Brainres.2006.04.044 |
0.319 |
|
| 2006 |
Karamboulas C, Dakubo GD, Liu J, De Repentigny Y, Yutzey K, Wallace VA, Kothary R, Skerjanc IS. Disruption of MEF2 activity in cardiomyoblasts inhibits cardiomyogenesis. Journal of Cell Science. 119: 4315-21. PMID 17003108 DOI: 10.1242/Jcs.03186 |
0.394 |
|
| 2006 |
Anderson CL, De Repentigny Y, Cifelli C, Marshall P, Renaud JM, Worton RG, Kothary R. The mouse dystrophin muscle promoter/enhancer drives expression of mini-dystrophin in transgenic mdx mice and rescues the dystrophy in these mice. Molecular Therapy : the Journal of the American Society of Gene Therapy. 14: 724-34. PMID 16807118 DOI: 10.1016/J.Ymthe.2006.04.013 |
0.393 |
|
| 2006 |
Lee KK, de Repentigny Y, Saulnier R, Rippstein P, Macklin WB, Kothary R. Dominant-negative beta1 integrin mice have region-specific myelin defects accompanied by alterations in MAPK activity. Glia. 53: 836-44. PMID 16575886 DOI: 10.1002/Glia.20343 |
0.356 |
|
| 2006 |
Pool M, Rippstein P, McBride H, Kothary R. Trafficking of macromolecules and organelles in cultured Dystonia musculorum sensory neurons is normal. The Journal of Comparative Neurology. 494: 549-58. PMID 16374799 DOI: 10.1002/Cne.20815 |
0.424 |
|
| 2006 |
Ichikawa H, De Repentigny Y, Kothary R, Sugimoto T. The survival of vagal and glossopharyngeal sensory neurons is dependent upon dystonin. Neuroscience. 137: 531-6. PMID 16289886 DOI: 10.1016/J.Neuroscience.2005.08.081 |
0.378 |
|
| 2006 |
Young KG, Pinheiro B, Kothary R. A Bpag1 isoform involved in cytoskeletal organization surrounding the nucleus. Experimental Cell Research. 312: 121-34. PMID 16289082 DOI: 10.1016/J.Yexcr.2005.10.002 |
0.354 |
|
| 2005 |
Pool M, Boudreau Larivière C, Bernier G, Young KG, Kothary R. Genetic alterations at the Bpag1 locus in dt mice and their impact on transcript expression. Mammalian Genome : Official Journal of the International Mammalian Genome Society. 16: 909-17. PMID 16341670 DOI: 10.1007/S00335-005-0073-4 |
0.406 |
|
| 2005 |
Shafey D, Côté PD, Kothary R. Hypomorphic Smn knockdown C2C12 myoblasts reveal intrinsic defects in myoblast fusion and myotube morphology. Experimental Cell Research. 311: 49-61. PMID 16219305 DOI: 10.1016/J.Yexcr.2005.08.019 |
0.423 |
|
| 2005 |
Stocksley MA, Chakkalakal JV, Bradford A, Miura P, De Repentigny Y, Kothary R, Jasmin BJ. A 1.3 kb promoter fragment confers spatial and temporal expression of utrophin A mRNA in mouse skeletal muscle fibers. Neuromuscular Disorders : Nmd. 15: 437-49. PMID 15907291 DOI: 10.1016/J.Nmd.2005.03.008 |
0.369 |
|
| 2005 |
Young KG, Kothary R. Spectrin repeat proteins in the nucleus. Bioessays : News and Reviews in Molecular, Cellular and Developmental Biology. 27: 144-52. PMID 15666356 DOI: 10.1002/Bies.20177 |
0.307 |
|
| 2005 |
Horsford DJ, Nguyen MT, Sellar GC, Kothary R, Arnheiter H, McInnes RR. Chx10 repression of Mitf is required for the maintenance of mammalian neuroretinal identity. Development (Cambridge, England). 132: 177-87. PMID 15576400 DOI: 10.1242/Dev.01571 |
0.349 |
|
| 2004 |
De Repentigny Y, Marshall P, Worton RG, Kothary R. The mouse dystrophin muscle enhancer-1 imparts skeletal muscle, but not cardiac muscle, expression onto the dystrophin Purkinje promoter in transgenic mice. Human Molecular Genetics. 13: 2853-62. PMID 15385445 DOI: 10.1093/Hmg/Ddh305 |
0.339 |
|
| 2003 |
Young KG, Pool M, Kothary R. Bpag1 localization to actin filaments and to the nucleus is regulated by its N-terminus. Journal of Cell Science. 116: 4543-55. PMID 14576348 DOI: 10.1242/Jcs.00764 |
0.336 |
|
| 2003 |
De Repentigny Y, Deschênes-Furry J, Jasmin BJ, Kothary R. Impaired fast axonal transport in neurons of the sciatic nerves from dystonia musculorum mice. Journal of Neurochemistry. 86: 564-71. PMID 12859670 DOI: 10.1046/J.1471-4159.2003.01861.X |
0.352 |
|
| 2003 |
DiDonato CJ, Parks RJ, Kothary R. Development of a gene therapy strategy for the restoration of survival motor neuron protein expression: implications for spinal muscular atrophy therapy. Human Gene Therapy. 14: 179-88. PMID 12614569 DOI: 10.1089/104303403321070874 |
0.428 |
|
| 2003 |
Lee SH, Zafer A, de Repentigny Y, Kothary R, Tremblay ML, Gros P, Duplay P, Webb JR, Vidal SM. Transgenic expression of the activating natural killer receptor Ly49H confers resistance to cytomegalovirus in genetically susceptible mice. The Journal of Experimental Medicine. 197: 515-26. PMID 12591908 DOI: 10.1084/Jem.20021713 |
0.333 |
|
| 2002 |
Boudreau-Larivière C, Kothary R. Differentiation potential of primary myogenic cells derived from skeletal muscle of dystonia musculorum mice. Differentiation; Research in Biological Diversity. 70: 247-56. PMID 12190986 DOI: 10.1046/J.1432-0436.2002.700603.X |
0.403 |
|
| 2002 |
Campsall KD, Mazerolle CJ, De Repentingy Y, Kothary R, Wallace VA. Characterization of transgene expression and Cre recombinase activity in a panel of Thy-1 promoter-Cre transgenic mice. Developmental Dynamics : An Official Publication of the American Association of Anatomists. 224: 135-43. PMID 12112467 DOI: 10.1002/Dvdy.10092 |
0.412 |
|
| 2002 |
Saulnier R, De Repentigny Y, Yong VW, Kothary R. Alterations in myelination in the central nervous system of dystonia musculorum mice. Journal of Neuroscience Research. 69: 233-42. PMID 12111805 DOI: 10.1002/Jnr.10289 |
0.359 |
|
| 2002 |
Marshall P, Chartrand N, De Repentigny Y, Kothary R, Pelletier L, Mueller R, Worton RG. Mouse dystrophin enhancer preferentially targets lacZ expression in skeletal and cardiac muscle. Developmental Dynamics : An Official Publication of the American Association of Anatomists. 224: 30-8. PMID 11984871 DOI: 10.1002/Dvdy.10080 |
0.369 |
|
| 2002 |
Bérubé NG, Jagla M, Smeenk C, De Repentigny Y, Kothary R, Picketts DJ. Neurodevelopmental defects resulting from ATRX overexpression in transgenic mice. Human Molecular Genetics. 11: 253-61. PMID 11823444 DOI: 10.1093/Hmg/11.3.253 |
0.398 |
|
| 2001 |
DiDonato CJ, Lorson CL, De Repentigny Y, Simard L, Chartrand C, Androphy EJ, Kothary R. Regulation of murine survival motor neuron (Smn) protein levels by modifying Smn exon 7 splicing. Human Molecular Genetics. 10: 2727-36. PMID 11726560 DOI: 10.1093/Hmg/10.23.2727 |
0.419 |
|
| 2001 |
De Repentigny Y, Côté PD, Pool M, Bernier G, Girard S, Vidal SM, Kothary R. Pathological and genetic analysis of the degenerating muscle (dmu) mouse: a new allele of Scn8a. Human Molecular Genetics. 10: 1819-27. PMID 11532991 DOI: 10.1093/Hmg/10.17.1819 |
0.413 |
|
| 2001 |
Carlsten JA, Kothary R, Wright DE. Glial cell line-derived neurotrophic factor-responsive and neurotrophin-3-responsive neurons require the cytoskeletal linker protein dystonin for postnatal survival. The Journal of Comparative Neurology. 432: 155-68. PMID 11241383 DOI: 10.1002/Cne.1094 |
0.426 |
|
| 2000 |
Mei Q, Kothary R, Wall L. The tkNeo gene, but not the pgkPuro gene, can influence the ability of the beta-globin LCR to enhance and confer position-independent expression onto the beta-globin gene. Experimental Cell Research. 260: 304-12. PMID 11035925 DOI: 10.1006/Excr.2000.5030 |
0.3 |
|
| 2000 |
Bernier G, Pool M, Kilcup M, Alfoldi J, De Repentigny Y, Kothary R. Acf7 (MACF) is an actin and microtubule linker protein whose expression predominates in neural, muscle, and lung development. Developmental Dynamics : An Official Publication of the American Association of Anatomists. 219: 216-25. PMID 11002341 DOI: 10.1002/1097-0177(2000)9999:9999<::Aid-Dvdy1041>3.0.Co;2-O |
0.377 |
|
| 2000 |
Mainguy G, Montesinos ML, Lesaffre B, Zevnik B, Karasawa M, Kothary R, Wurst W, Prochiantz A, Volovitch M. An induction gene trap for identifying a homeoprotein-regulated locus. Nature Biotechnology. 18: 746-9. PMID 10888842 DOI: 10.1038/77312 |
0.348 |
|
| 1999 |
Kolodziejczyk SM, Wang L, Balazsi K, DeRepentigny Y, Kothary R, Megeney LA. MEF2 is upregulated during cardiac hypertrophy and is required for normal post-natal growth of the myocardium. Current Biology : Cb. 9: 1203-6. PMID 10531040 DOI: 10.1016/S0960-9822(00)80027-5 |
0.311 |
|
| 1999 |
Dalpé G, Mathieu M, Comtois A, Zhu E, Wasiak S, De Repentigny Y, Leclerc N, Kothary R. Dystonin-deficient mice exhibit an intrinsic muscle weakness and an instability of skeletal muscle cytoarchitecture. Developmental Biology. 210: 367-80. PMID 10357897 DOI: 10.1006/Dbio.1999.9263 |
0.386 |
|
| 1998 |
Krause R, Hemberger M, Messerschmid M, Mayer W, Kothary R, Dixkens C, Fundele R. Molecular cloning and characterization of murine Mpgc60, a gene predominantly expressed in the intestinal tract. Differentiation; Research in Biological Diversity. 63: 285-94. PMID 9810707 DOI: 10.1046/J.1432-0436.1998.6350285.X |
0.33 |
|
| 1998 |
Dalpé G, Leclerc N, Vallée A, Messer A, Mathieu M, De Repentigny Y, Kothary R. Dystonin Is Essential for Maintaining Neuronal Cytoskeleton Organization. Molecular and Cellular Neurosciences. 10: 243-57. PMID 9618216 DOI: 10.1006/Mcne.1997.0660 |
0.393 |
|
| 1998 |
Bernier G, Kothary R. Prenatal onset of axonopathy in Dystonia musculorum mice. Developmental Genetics. 22: 160-8. PMID 9581287 DOI: 10.1002/(Sici)1520-6408(1998)22:2<160::Aid-Dvg5>3.0.Co;2-4 |
0.352 |
|
| 1997 |
Guy LG, Kothary R, Wall L. Position effects in mice carrying a lacZ transgene in cis with the beta-globin LCR can be explained by a graded model. Nucleic Acids Research. 25: 4400-7. PMID 9336475 DOI: 10.1093/Nar/25.21.4400 |
0.323 |
|
| 1996 |
Bernier G, Mathieu M, De Repentigny Y, Vidal SM, Kothary R. Cloning and characterization of mouse ACF7, a novel member of the dystonin subfamily of actin binding proteins. Genomics. 38: 19-29. PMID 8954775 DOI: 10.1006/Geno.1996.0587 |
0.386 |
|
| 1996 |
Guy LG, Kothary R, DeRepentigny Y, Delvoye N, Ellis J, Wall L. The beta-globin locus control region enhances transcription of but does not confer position-independent expression onto the lacZ gene in transgenic mice. The Embo Journal. 15: 3713-21. PMID 8670875 DOI: 10.1002/J.1460-2075.1996.Tb00741.X |
0.344 |
|
| 1995 |
Bernier G, Brown A, Dalpé G, De Repentigny Y, Mathieu M, Kothary R. Dystonin expression in the developing nervous system predominates in the neurons that degenerate in dystonia musculorum mutant mice. Molecular and Cellular Neurosciences. 6: 509-20. PMID 8742268 DOI: 10.1006/Mcne.1995.0003 |
0.456 |
|
| 1995 |
Bernier G, Brown A, Dalpé G, Mathieu M, De Repentigny Y, Kothary R. Dystonin transcripts are altered and their levels are reduced in the mouse neurological mutant dt24J Biochemistry and Cell Biology = Biochimie Et Biologie Cellulaire. 73: 605-609. PMID 8714679 DOI: 10.1139/O95-066 |
0.386 |
|
| 1995 |
Brown A, Dalpé G, Mathieu M, Kothary R. Cloning and characterization of the neural isoforms of human dystonin. Genomics. 29: 777-80. PMID 8575775 DOI: 10.1006/Geno.1995.9936 |
0.345 |
|
| 1995 |
Brown A, Bernier G, Mathieu M, Rossant J, Kothary R. The mouse dystonia musculorum gene is a neural isoform of bullous pemphigoid antigen 1. Nature Genetics. 10: 301-6. PMID 7670468 DOI: 10.1038/Ng0795-301 |
0.564 |
|
| 1995 |
Sévigny G, Kothary R, Tremblay E, De Repentigny Y, Joly EC, Bibor-Hardy V. The cytosolic chaperonin subunit TRiC-P5 begins to be expressed at the two-cell stage in mouse embryos. Biochemical and Biophysical Research Communications. 216: 279-83. PMID 7488101 DOI: 10.1006/Bbrc.1995.2621 |
0.31 |
|
| 1994 |
Brown A, Copeland NG, Gilbert DJ, Jenkins NA, Rossant J, Kothary R. The genomic structure of an insertional mutation in the dystonia musculorum locus. Genomics. 20: 371-6. PMID 8034309 DOI: 10.1006/Geno.1994.1190 |
0.48 |
|
| 1994 |
Brown A, Lemieux N, Rossant J, Kothary R. Human homolog of a mouse sequence from the dystonia musculorum locus is on chromosome 6p12. Mammalian Genome : Official Journal of the International Mammalian Genome Society. 5: 434-7. PMID 7919656 DOI: 10.1007/Bf00357004 |
0.498 |
|
| 1993 |
Franz T, Kothary R, Surani MA, Halata Z, Grim M. The Splotch mutation interferes with muscle development in the limbs. Anatomy and Embryology. 187: 153-60. PMID 8238963 DOI: 10.1007/Bf00171747 |
0.373 |
|
| 1991 |
Singh PB, Miller JR, Pearce J, Kothary R, Burton RD, Paro R, James TC, Gaunt SJ. A sequence motif found in a Drosophila heterochromatin protein is conserved in animals and plants. Nucleic Acids Research. 19: 789-94. PMID 1708124 DOI: 10.1093/Nar/19.4.789 |
0.312 |
|
| 1991 |
Kothary R, Barton SC, Franz T, Norris ML, Hettle S, Surani MA. Unusual cell specific expression of a major human cytomegalovirus immediate early gene promoter-lacZ hybrid gene in transgenic mouse embryos. Mechanisms of Development. 35: 25-31. PMID 1659441 DOI: 10.1016/0925-4773(91)90038-8 |
0.367 |
|
| 1989 |
Kothary R, Clapoff S, Darling S, Perry MD, Moran LA, Rossant J. Inducible expression of an hsp68-lacZ hybrid gene in transgenic mice. Development (Cambridge, England). 105: 707-14. PMID 2557196 |
0.466 |
|
| 1988 |
Varmuza S, Prideaux V, Kothary R, Rossant J. Polytene chromosomes in mouse trophoblast giant cells. Development (Cambridge, England). 102: 127-34. PMID 3166422 |
0.387 |
|
| 1988 |
Kothary R, Clapoff S, Brown A, Campbell R, Peterson A, Rossant J. A transgene containing lacZ inserted into the dystonia locus is expressed in neural tube. Nature. 335: 435-7. PMID 3138544 DOI: 10.1038/335435A0 |
0.53 |
|
| 1987 |
Kothary R, Perry MD, Moran LA, Rossant J. Cell-lineage-specific expression of the mouse hsp68 gene during embryogenesis. Developmental Biology. 121: 342-8. PMID 3582732 DOI: 10.1016/0012-1606(87)90170-9 |
0.496 |
|
| 1985 |
Huszar D, Balling R, Kothary R, Magli MC, Hozumi N, Rossant J, Bernstein A. Insertion of a bacterial gene into the mouse germ line using an infectious retrovirus vector. Proceedings of the National Academy of Sciences of the United States of America. 82: 8587-91. PMID 2417225 DOI: 10.1073/Pnas.82.24.8587 |
0.538 |
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