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Results: 1 to 20 of 130


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.

McFall ER, Murray LM, Lunde JA, Jasmin BJ, Kothary R, Parks RJ.

Virology. 2014 Nov;468-470:444-53. doi: 10.1016/j.virol.2014.08.026. Epub 2014 Sep 20.


The Smn-independent beneficial effects of trichostatin A on an intermediate mouse model of spinal muscular atrophy.

Liu H, Yazdani A, Murray LM, Beauvais A, Kothary R.

PLoS One. 2014 Jul 1;9(7):e101225. doi: 10.1371/journal.pone.0101225. eCollection 2014.


Snf2h-mediated chromatin organization and histone H1 dynamics govern cerebellar morphogenesis and neural maturation.

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, Majumder R, Ioshikhes I, Wallace VA, Kothary R, Meshorer E, Stopka T, Skoultchi AI, Picketts DJ.

Nat Commun. 2014 Jun 20;5:4181. doi: 10.1038/ncomms5181.


Biochemical and cellular analysis of human variants of the DYT1 dystonia protein, TorsinA/TOR1A.

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.

Hum Mutat. 2014 Sep;35(9):1101-13. doi: 10.1002/humu.22602. Epub 2014 Jul 17.


Myogenic program dysregulation is contributory to disease pathogenesis in spinal muscular atrophy.

Boyer JG, Deguise MO, Murray LM, Yazdani A, De Repentigny Y, Boudreau-Larivière C, Kothary R.

Hum Mol Genet. 2014 Aug 15;23(16):4249-59. doi: 10.1093/hmg/ddu142. Epub 2014 Apr 1.


Defects in pancreatic development and glucose metabolism in SMN-depleted mice independent of canonical spinal muscular atrophy neuromuscular pathology.

Bowerman M, Michalski JP, Beauvais A, Murray LM, DeRepentigny Y, Kothary R.

Hum Mol Genet. 2014 Jul 1;23(13):3432-44. doi: 10.1093/hmg/ddu052. Epub 2014 Feb 4.


Dissection of the transversus abdominis muscle for whole-mount neuromuscular junction analysis.

Murray L, Gillingwater TH, Kothary R.

J Vis Exp. 2014 Jan 11;(83):e51162. doi: 10.3791/51162.


More than a bystander: the contributions of intrinsic skeletal muscle defects in motor neuron diseases.

Boyer JG, Ferrier A, Kothary R.

Front Physiol. 2013 Dec 18;4:356. doi: 10.3389/fphys.2013.00356. Review.


Transgenic expression of neuronal dystonin isoform 2 partially rescues the disease phenotype of the dystonia musculorum mouse model of hereditary sensory autonomic neuropathy VI.

Ferrier A, Sato T, De Repentigny Y, Gibeault S, Bhanot K, O'Meara RW, Lynch-Godrei A, Kornfeld SF, Young KG, Kothary R.

Hum Mol Genet. 2014 May 15;23(10):2694-710. doi: 10.1093/hmg/ddt663. Epub 2013 Dec 30.


Early onset muscle weakness and disruption of muscle proteins in mouse models of spinal muscular atrophy.

Boyer JG, Murray LM, Scott K, De Repentigny Y, Renaud JM, Kothary R.

Skelet Muscle. 2013 Oct 11;3(1):24. doi: 10.1186/2044-5040-3-24.


Supraphysiological expression of survival motor neuron protein from an adenovirus vector does not adversely affect cell function.

Goulet BB, McFall ER, Wong CM, Kothary R, Parks RJ.

Biochem Cell Biol. 2013 Aug;91(4):252-64. doi: 10.1139/bcb-2012-0094. Epub 2013 Apr 8.


Six1 regulates MyoD expression in adult muscle progenitor cells.

Liu Y, Chakroun I, Yang D, Horner E, Liang J, Aziz A, Chu A, De Repentigny Y, Dilworth FJ, Kothary R, Blais A.

PLoS One. 2013 Jun 28;8(6):e67762. doi: 10.1371/journal.pone.0067762. Print 2013.


Distinct roles for Ste20-like kinase SLK in muscle function and regeneration.

Storbeck CJ, Al-Zahrani KN, Sriram R, Kawesa S, O'Reilly P, Daniel K, McKay M, Kothary R, Tsilfidis C, Sabourin LA.

Skelet Muscle. 2013 Jul 1;3(1):16. doi: 10.1186/2044-5040-3-16.


Integrin-linked kinase regulates process extension in oligodendrocytes via control of actin cytoskeletal dynamics.

O'Meara RW, Michalski JP, Anderson C, Bhanot K, Rippstein P, Kothary R.

J Neurosci. 2013 Jun 5;33(23):9781-93. doi: 10.1523/JNEUROSCI.5582-12.2013.


Cellular and molecular biology of neuronal dystonin.

Ferrier A, Boyer JG, Kothary R.

Int Rev Cell Mol Biol. 2013;300:85-120. doi: 10.1016/B978-0-12-405210-9.00003-5. Review.


A novel function for the survival motoneuron protein as a translational regulator.

Sanchez G, Dury AY, Murray LM, Biondi O, Tadesse H, El Fatimy R, Kothary R, Charbonnier F, Khandjian EW, Côté J.

Hum Mol Genet. 2013 Feb 15;22(4):668-84. doi: 10.1093/hmg/dds474. Epub 2012 Nov 6.


Defects in neuromuscular junction remodelling in the Smn(2B/-) mouse model of spinal muscular atrophy.

Murray LM, Beauvais A, Bhanot K, Kothary R.

Neurobiol Dis. 2013 Jan;49:57-67. doi: 10.1016/j.nbd.2012.08.019. Epub 2012 Aug 30.


Glucose metabolism and pancreatic defects in spinal muscular atrophy.

Bowerman M, Swoboda KJ, Michalski JP, Wang GS, Reeks C, Beauvais A, Murphy K, Woulfe J, Screaton RA, Scott FW, Kothary R.

Ann Neurol. 2012 Aug;72(2):256-68. doi: 10.1002/ana.23582.

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