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Items: 1 to 50 of 163

1.

Characterization of gastrointestinal pathologies in the dystonia musculorum mouse model for hereditary sensory and autonomic neuropathy type VI.

Lynch-Godrei A, De Repentigny Y, Yaworski RA, Gagnon S, Butcher J, Manoogian J, Stintzi A, Kothary R.

Neurogastroenterol Motil. 2019 Dec 9:e13773. doi: 10.1111/nmo.13773. [Epub ahead of print]

PMID:
31814231
2.

Snf2h Drives Chromatin Remodeling to Prime Upper Layer Cortical Neuron Development.

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.

Front Mol Neurosci. 2019 Oct 17;12:243. doi: 10.3389/fnmol.2019.00243. eCollection 2019.

3.

Low fat diets increase survival of a mouse model of spinal muscular atrophy.

Deguise MO, Chehade L, Tierney A, Beauvais A, Kothary R.

Ann Clin Transl Neurol. 2019 Nov;6(11):2340-2346. doi: 10.1002/acn3.50920. Epub 2019 Oct 13.

4.

Impaired kidney structure and function in spinal muscular atrophy.

Nery FC, Siranosian JJ, Rosales I, Deguise MO, Sharma A, Muhtaseb AW, Nwe P, Johnstone AJ, Zhang R, Fatouraei M, Huemer N, Alves CRR, Kothary R, Swoboda KJ.

Neurol Genet. 2019 Aug 12;5(5):e353. doi: 10.1212/NXG.0000000000000353. eCollection 2019 Oct.

5.

Inhibitory milieu at the multiple sclerosis lesion site and the challenges for remyelination.

Galloway DA, Gowing E, Setayeshgar S, Kothary R.

Glia. 2019 Aug 23. doi: 10.1002/glia.23711. [Epub ahead of print] Review.

PMID:
31441132
6.

Abnormal fatty acid metabolism is a core component of spinal muscular atrophy.

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, Kubinski S, Claus P, Murray LM, Bowerman M, Gillingwater TH, Bertoli S, Parson SH, Kothary R.

Ann Clin Transl Neurol. 2019 Aug;6(8):1519-1532. doi: 10.1002/acn3.50855. Epub 2019 Jul 26.

7.

Pathologic Alterations in the Proteome of Synaptosomes from a Mouse Model of Spinal Muscular Atrophy.

Eshraghi M, Gombar R, De Repentigny Y, Vacratsis PO, Kothary R.

J Proteome Res. 2019 Aug 2;18(8):3042-3051. doi: 10.1021/acs.jproteome.9b00159. Epub 2019 Jul 11.

PMID:
31262178
8.

Report on the 4th Ottawa International Conference on Neuromuscular Disease and Biology - September 5-7, 2017, Ottawa, Canada.

Chardon JW, Jasmin BJ, Kothary R, Parks RJ.

J Neuromuscul Dis. 2018;5(4):539-552. doi: 10.3233/JND-180353. No abstract available.

PMID:
30373960
9.

Surgical Artificial Insemination in Mice.

De Repentigny Y, Kothary R.

Cold Spring Harb Protoc. 2018 Sep 4;2018(9). doi: 10.1101/pdb.prot092734.

PMID:
30181219
10.

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.

Lynch-Godrei A, De Repentigny Y, Gagnon S, Trung MT, Kothary R.

Hum Mol Genet. 2018 Oct 15;27(20):3598-3611. doi: 10.1093/hmg/ddy250.

PMID:
29982604
11.

Interventions Targeting Glucocorticoid-Krüppel-like Factor 15-Branched-Chain Amino Acid Signaling Improve Disease Phenotypes in Spinal Muscular Atrophy Mice.

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, Jain MK, Gillingwater TH, Claus P, Kothary R, Wood MJA, Bowerman M.

EBioMedicine. 2018 May;31:226-242. doi: 10.1016/j.ebiom.2018.04.024. Epub 2018 May 4.

12.

Pathogenic commonalities between spinal muscular atrophy and amyotrophic lateral sclerosis: Converging roads to therapeutic development.

Bowerman M, Murray LM, Scamps F, Schneider BL, Kothary R, Raoul C.

Eur J Med Genet. 2018 Nov;61(11):685-698. doi: 10.1016/j.ejmg.2017.12.001. Epub 2017 Dec 5. Review.

PMID:
29313812
13.

Survival Motor Neuron Protein is Released from Cells in Exosomes: A Potential Biomarker for Spinal Muscular Atrophy.

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.

Sci Rep. 2017 Oct 24;7(1):13859. doi: 10.1038/s41598-017-14313-z.

14.

Splicing arrays reveal novel RBM10 targets, including SMN2 pre-mRNA.

Sutherland LC, Thibault P, Durand M, Lapointe E, Knee JM, Beauvais A, Kalatskaya I, Hunt SC, Loiselle JJ, Roy JG, Tessier SJ, Ybazeta G, Stein L, Kothary R, Klinck R, Chabot B.

BMC Mol Biol. 2017 Jul 20;18(1):19. doi: 10.1186/s12867-017-0096-x.

15.

New insights into SMA pathogenesis: immune dysfunction and neuroinflammation.

Deguise MO, Kothary R.

Ann Clin Transl Neurol. 2017 May 18;4(7):522-530. doi: 10.1002/acn3.423. eCollection 2017 Jul.

16.

Effect of genetic background on the phenotype of the Smn2B/- mouse model of spinal muscular atrophy.

Eshraghi M, McFall E, Gibeault S, Kothary R.

Hum Mol Genet. 2016 Oct 15;25(20):4494-4506. doi: 10.1093/hmg/ddw278.

17.

Immune dysregulation may contribute to disease pathogenesis in spinal muscular atrophy mice.

Deguise MO, De Repentigny Y, McFall E, Auclair N, Sad S, Kothary R.

Hum Mol Genet. 2017 Feb 15;26(4):801-819. doi: 10.1093/hmg/ddw434.

18.

Oligodendrocyte development and CNS myelination are unaffected in a mouse model of severe spinal muscular atrophy.

O'Meara RW, Cummings SE, De Repentigny Y, McFall E, Michalski JP, Deguise MO, Gibeault S, Kothary R.

Hum Mol Genet. 2017 Jan 15;26(2):282-292. doi: 10.1093/hmg/ddw385.

PMID:
28069797
19.

Spinal Muscular Atrophy: More than a Disease of Motor Neurons?

Nash LA, Burns JK, Chardon JW, Kothary R, Parks RJ.

Curr Mol Med. 2016;16(9):779-792. doi: 10.2174/1566524016666161128113338. Review.

PMID:
27894243
20.

Report on the 3rd Ottawa International Conference on Neuromuscular Biology, Disease and Therapy - September 24-26, 2015, Ottawa, Canada.

Chardon JW, Jasmin BJ, Kothary R, Parks RJ.

J Neuromuscul Dis. 2016 Aug 30;3(3):431-442. No abstract available.

21.

Voluntary Running Triggers VGF-Mediated Oligodendrogenesis to Prolong the Lifespan of Snf2h-Null Ataxic Mice.

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.

Cell Rep. 2016 Oct 11;17(3):862-875. doi: 10.1016/j.celrep.2016.09.030.

22.

Opening the window: The case for carrier and perinatal screening for spinal muscular atrophy.

Burns JK, Kothary R, Parks RJ.

Neuromuscul Disord. 2016 Sep;26(9):551-9. doi: 10.1016/j.nmd.2016.06.459. Epub 2016 Jun 23. Review.

23.

Differential induction of muscle atrophy pathways in two mouse models of spinal muscular atrophy.

Deguise MO, Boyer JG, McFall ER, Yazdani A, De Repentigny Y, Kothary R.

Sci Rep. 2016 Jun 28;6:28846. doi: 10.1038/srep28846.

24.

Corrigendum: Establishment of a cone photoreceptor transplantation platform based on a novel cone-GFP reporter mouse line.

Smiley S, Nickerson PE, Comanita L, Daftarian N, El-Sehemy A, Tsai EL, Matan-Lithwick S, Yan K, Thurig S, Touahri Y, Dixit R, Aavani T, De Repentigny Y, Baker A, Tsilfidis C, Biernaskie J, Sauvé Y, Schuurmans C, Kothary R, Mears AJ, Wallace VA.

Sci Rep. 2016 Apr 22;6:24012. doi: 10.1038/srep24012. No abstract available.

25.

Establishment of a cone photoreceptor transplantation platform based on a novel cone-GFP reporter mouse line.

Smiley S, Nickerson PE, Comanita L, Daftarian N, El-Sehemy A, Tsai EL, Matan-Lithwick S, Yan K, Thurig S, Touahri Y, Dixit R, Aavani T, De Repentingy Y, Baker A, Tsilfidis C, Biernaskie J, Sauvé Y, Schuurmans C, Kothary R, Mears AJ, Wallace VA.

Sci Rep. 2016 Mar 11;6:22867. doi: 10.1038/srep22867. Erratum in: Sci Rep. 2016 Apr 22;6:24012. De Repentigny, Yves [corrected to De Repentingy, Yves].

26.

Cytoskeletal Linker Protein Dystonin Is Not Critical to Terminal Oligodendrocyte Differentiation or CNS Myelination.

Kornfeld SF, Lynch-Godrei A, Bonin SR, Gibeault S, De Repentigny Y, Kothary R.

PLoS One. 2016 Feb 17;11(2):e0149201. doi: 10.1371/journal.pone.0149201. eCollection 2016.

27.

A new in vitro mouse oligodendrocyte precursor cell migration assay reveals a role for integrin-linked kinase in cell motility.

O'Meara RW, Cummings SE, Michalski JP, Kothary R.

BMC Neurosci. 2016 Feb 1;17:7. doi: 10.1186/s12868-016-0242-2.

28.

Functional and Genetic Analysis of Neuronal Isoforms of BPAG1.

Lynch-Godrei A, Kothary R.

Methods Enzymol. 2016;569:355-72. doi: 10.1016/bs.mie.2015.05.004. Epub 2015 Jun 1. Review.

PMID:
26778567
29.

Integrin-linked kinase regulates oligodendrocyte cytoskeleton, growth cone, and adhesion dynamics.

Michalski JP, Cummings SE, O'Meara RW, Kothary R.

J Neurochem. 2016 Feb;136(3):536-49. doi: 10.1111/jnc.13446. Epub 2016 Jan 11.

30.

Oligodendrocytes in a Nutshell.

Michalski JP, Kothary R.

Front Cell Neurosci. 2015 Sep 1;9:340. doi: 10.3389/fncel.2015.00340. eCollection 2015. Review.

31.

Transcriptional profiling of differentially vulnerable motor neurons at pre-symptomatic stage in the Smn (2b/-) mouse model of spinal muscular atrophy.

Murray LM, Beauvais A, Gibeault S, Courtney NL, Kothary R.

Acta Neuropathol Commun. 2015 Sep 15;3:55. doi: 10.1186/s40478-015-0231-1.

32.

Disruption in the autophagic process underlies the sensory neuropathy in dystonia musculorum mice.

Ferrier A, De Repentigny Y, Lynch-Godrei A, Gibeault S, Eid W, Kuo D, Zha X, Kothary R.

Autophagy. 2015;11(7):1025-36. doi: 10.1080/15548627.2015.1052207.

33.

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;468-470:444-453. doi: 10.1016/j.virol.2014.08.026. Epub 2014 Sep 20.

34.

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.

35.

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.

36.

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.

37.

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.

38.

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.

39.

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.

40.

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.

41.

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.

42.

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.

43.

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.

PMID:
23859020
44.

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.

45.

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.

46.

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.

47.
48.

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.

PMID:
23273860
49.

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.

PMID:
23136128
50.

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.

PMID:
22960106

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