Rashmi Kothary, PhD

rkothary@ohri.ca
Tel: 613-737-8707
Lab Tel: 613-739-6634
Heidi Hickey (Research Administrative Assistant)
Research Administration Tel: 613-737-8899 x78769
Fax: 613-737-8803


COMPLETE LIST OF PUBLICATIONS

  1. Kothary, R.K., and Candido, E.P.M. (1982). Induction of a novel set of polypeptides by heat shock or sodium arsenite in cultured cells of rainbow trout, Salmo gairdnerri. Can. J. Biochem. 60, 347-355.

  2. Kothary, R.K., Jones, D., and Candido, E.P.M. (1984). The 70kD heat shock polypeptides from rainbow trout: characterization of cDNA sequences. Mol. Cell. Biol. 4, 1785-1791.

  3. Kothary, R.K., Burgess, E.A., and Candido, E.P.M. (1984). The heat shock phenomenon in cultured cells of the rainbow trout: Hsp70 mRNA synthesis and turnover. Biochim. Biophys. Acta. 783, 137-143.

  4. Burgess, E.A., Kothary, R.K., and Candido, E.P.M. (1984). Inducibility of heat shock polypeptides in cells containing hyperacetylated histones. Exp. Cell Res. 155, 273-277.

  5. Kothary, R.K., Jones, D., and Candido, E.P.M. (1985). IS186: an Escherichia coli insertion element isolated from a cDNA library. J. Bacteriology 164, 957-959.

  6. Huszar, D., Balling, R., Kothary, R.K., Magli, M.C., Hozumi, N., Rossant, J., and Bernstein, A. (1985). Insertion of a bacterial gene into the mouse germ line using an infectious retrovirus vector. Proc.  natn. Acad. Sci. U.S.A. 82, 8587-8591.

  7. Kothary, R.K., Perry, M.D., Moran, L.A., and Rossant, J. (1987). Cell lineage specific expression of the mouse hsp68 gene during embryogenesis. Dev. Biol. 121, 342-348.

  8. Varmuza, S., Prideaux, V., Kothary, R.K., and Rossant, J. (1988). Polytene chromosomes in mouse trophoblast giant cells. Development 102, 127-134.

  9. Kothary, R.K., Clapoff, S., Brown, A., Campbell, R., Peterson, A., and Rossant, J. (1988). A transgene containing lacZ inserted into the dystonia locus is expressed in neural tube. Nature (London) 335, 435-437.

  10. Kothary, R.K., Clapoff, S., Darling, S., Perry, M.D., Moran, L.A., and Rossant, J. (1989). Inducible expression of an hsp68-lacZ hybrid gene in transgenic mice. Development 105, 707-714.

  11. Kothary, R.K., Allen, N.D., and Surani, M.A.H. (1989). Transgenes as molecular probes of mammalian developmental genetics. In Oxford Surveys on Eukaryotic Genes. vol. 6 (ed. N. Maclean), pp. 145-178. Oxford University Press, Oxford.

  12. Kothary, R.K. (1989). Use of transgenes to identify developmentally regulated loci in the mouse genome. In In Vitro Approaches to Mammalian Gamete Maturation and Embryonic Development. (eds. A. Lauria and F. Gandolfi), pp. 63-69. Christengraf, Rome.

  13. Surani, M.A., Kothary, R.K., Allen, N.D., Singh, P.B., Fundele, R., Ferguson-Smith, A.C., and Barton, S.C. (1990). Genome imprinting and development in the mouse. Development (Suppl.) 110, 89-98.

  14. Singh, P.B., Miller, J.R., Pearce, J., Kothary, R.K., Burton, R.D., Paro, R., James, T.C., and Gaunt, S.J. (1991). A sequence motif found in a Drosophila heterochromatin protein is conserved in animals and plants. Nucleic Acids Res. 19, 789-794.

  15. Kothary, R.K., Barton, S.C., Franz, T., Norris, M.L., Hettle, S., and Surani, M.A.H. (1991). An 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.

  16. Fundele, R.F., Howlett, S.K., Kothary, R.K., Mills, W.E., and Surani, M.A. (1991). Developmental potential of parthenogenetic cells: Role of genotype-specific modifiers. Development 113, 941-946.

  17. Jagerbauer, E-M., Fraser, A., Herbst, E.W., Kothary, R.K., and Fundele, R.F. (1992). Parthenogenetic stem cells in postnatal mouse chimeras. Development 116, 95-102.

  18. Kothary, R.K., Allen, N.D., Barton, S.C., Norris, M., and Surani, M.A. (1992). Factors affecting cellular mosaicism in the expression of a lacZ transgene in two-cell stage mouse embryos. Biochem. Cell Biol. 70, 1097-1104.

  19. Franz, T. and Kothary, R.K. (1993). Characterization of the neural crest defect in Splotch (Sp1H) mutant mice using a lacZ transgene. Developmental Brain Research 72, 99-105.

  20. Franz, T., Kothary, R., Surani, M.A.H., Halata, Z., and Grim, M. (1993). The Splotch mutation interferes with muscle development in the limbs. Anat. Embryol. 187, 153-160.

  21. Brown, A.B., Copeland, N., Gilbert, D.J., Jenkins, N.A., Rossant, J., and Kothary, R.K. (1994). The genomic structure of an insertional mutation in the dystonia musculorum locus. Genomics 20, 371-376.

  22. Brown, A.B., Lemieux, N., Rossant, J., and Kothary, R.K. (1994). Human homolog of a mouse sequence from the dystonia musculorum locus is on Chromosome 6p12. Mammalian Genome 5, 434-437.

  23. Bérard, J., Gaboury, L., Landers, M., De Repentigny, Y., Houle, B., Kothary, R., and Bradley, W.E.C. (1994). Hyperplasia and tumors in lung, breast and other tissues in mice carrying a RARß4-like transgene. EMBO J. 13, 5570-5580.

  24. Bender, R., Surani, M.A.H., Kothary, R., Li, L-L., Fürst, D.O., Christ, B., and Fundele, R. (1995). Tissue specific loss of proliferative capacity of parthenogenetic cells in fetal mouse chimeras. Roux's Archives of Developmental Biology 204, 436-443.

  25. Brown, A., Bernier, G., Mathieu, M., Rossant, J., and Kothary, R. (1995). The mouse dystonia musculorum gene is a neural isoform of bullous pemphigoid antigen 1. Nature Genetics 10, 301-306.

  26. Brown, A., Dalpé, G., Mathieu, M., and Kothary, R. (1995). Cloning and characterization of the neural isoforms of human dystonin. Genomics 29, 777-780.

  27. Sévigny, G., Kothary, R., Tremblay, E., De Repentigny, Y., Joly, E.C., and Bibor-Hardy, V. (1995). The cytosolic chaperonin subunit TRiC-P5 begins to be expressed at the two-cell stage in mouse embryos. BBRC 216, 279-283.

  28. Bernier, G., Brown, A., Dalpé, G., Mathieu, M., De Repentigny, Y., and Kothary, R. (1995). Dystonin transcripts are altered and their levels are reduced in the mouse neurological mutant dt24J. Biochem. Cell Biol. 73, 605-609.

  29. Bernier, G., Brown, A., Dalpé, G., De Repentigny, Y., Mathieu, M., and Kothary, R. (1995). Dystonin expression in the developing nervous system predominates in the neurons that degenerate in dystonia musculorum mutant mice. Mol. Cell. Neurosci. 6, 509-520.

  30. De Repentigny, Y., and Kothary, R. (1996). An improved method for the artificial insemination of mice - oviduct transfer of spermatozoa. Trends in Genetics 12, 44-45.

  31. Guy, L.-G., Kothary, R., De Repentigny, Y., Ellis, J., Delvoye, N., and Wall, L.A. (1996). The ß-globin locus control region enhances transcription of but does not confer position-independent expression onto the lacZ gene in transgenic mice. EMBO J. 15, 3713-3721.

  32. Bérard, J., Laboune, F., Mukuna, M., Massé, S., Kothary, R., and Bradley, W.E.C. (1996). Lung tumours in mice expressing an antisense RARß2 transgene. FASEB J. 10, 1091-1097.

  33. Bernier, G., Mathieu, M., De Repentigny, Y., Vidal, S., and Kothary, R.K. (1996). Cloning and characterization of mouse ACF7, a novel member of the dystonin subfamily of actin crosslinkers. Genomics 38, 19-29.

  34. Dagenais, A., Kothary, R., and Berthiaume, Y. (1997). The alpha subunit of the epithelial sodium channel in the mouse: developmental regulation of its expression. Pediatr. Res. 42, 327-334.

  35. Guy, L.-G., Kothary, R., and Wall, L.A. (1997). Position effects in mice carrying a lacZ transgene in cis with the ß-globin LCR can be explained by a graded model. Nuc. Acids Res. 21, 4400-4407.

  36. Dalpé, G., Leclerc, N., Vallée, A., Messer, A., Mathieu, M., De Repentigny, Y., and Kothary, R. (1998). Dystonin is essential for maintaining neuronal cytoskeleton organization. Mol. Cell. Neurosci. 10, 243-257.

  37. Bernier, G., and Kothary, R. (1998). Prenatal onset of axonopathy in dystonia musculorum mice. Dev. Gen. 22, 160-168.

  38. Bernier, G., De Repentigny, Y., Mathieu, M., David, S., and Kothary, R. (1998). Dystonin is an essential component of the Schwann cell cytoskeleton at the time of myelination. Development 125, 2135-2148.

  39. Krause, R., Hemberger, M., Messerschmid, M., Mayer, W., Kothary, R., Dixkens, C., and Fundele, R. (1998). Molecular cloning and characterization of murine Mpgc60, a gene predominantly expressed in the intestinal tract. Differentiation 63, 285-294.

  40. Dalpé, G., Mathieu, M., Comtois, A., Zhu, E., Wasiak, S., De Repentigny, Y., Leclerc, N., and Kothary, R. (1999). Dystonin-deficient mice exhibit an intrinsic muscle weakness and an instability of muscle cytoarchitecture. Developmental Biology 210, 367-380.

  41. Kolodziejczyk, S.M., Wang, L., Balaszi, K., De Repentigny, Y., Kothary, R., and Megeney, L.A. (1999). MEF2 is upregulated during cardiac hypertrophy and is required for normal post-natal growth of the myocardium. Current Biology 9, 1203-1206.

  42. Mainguy, G., Montesinos, M.L., Lesaffre, B., Zevnik, B., Karasawa, M., Kothary, R., Wurst, W., Prochiantz, A., and Volovitch, M. (2000). An induction gene trap for identifying a homeoprotein-regulated locus. Nature Biotech. 18, 746-749.

  43. Bernier, G., Pool, M., Kilcup, M., Alfoldi, J., De Repentigny, Y., and Kothary, R. (2000). Acf7 (MACF) is an actin and microtubule linker protein whose expression predominates in neural, muscle and lung development. Dev. Dyn. 219, 216-225.

  44. Mei, Q., Kothary, R., and Wall L. (2000). The tkNeo gene, but not the pgkPuro gene, can influence the ability of the ß-globin LCR to enhance and confer position-independent expression onto the ß-globin gene. Exp. Cell Res. 260, 304-312.

  45. Carlsten, J. A., Kothary, R., and Wright, D.E. (2001). GDNF- and NT-3-responsive neurons require the cytoskeletal linker protein dystonin for postnatal survival. Journal of Comparative Neurology 432, 155-168.

  46. De Repentigny Y., Côté P., Pool M., Bernier G., Girard S., Vidal S.M., and Kothary R., (2001).  Pathological and genetic analysis of the degenerating muscle (dmu) mouse:  a new allele of Scn8a.  Human Molecular Genetics, 10, 1819-1827.

  47. Dagenais, A., Denis, C., Vives, M.F., Girouard, S., Masse, C., Nguyen, T., Yamagata, T., Grygorcyzk, C., Kothary, R., and Berthiaume, Y. (2001).  Modulation of alpha-EnaC and alpha (1)-Na (+)-K(+)-ATPase by camp and dexamethasone in alveolar epithelial cells.  Am. J. Physiol. Lung Cell Mol. Physiol 28, L217-30.

  48. DiDonato, C., Lorson, C., De Repentigny, Y., Simard, L., Androphy, E., and Kothary, R. (2001). Regulation of murine Survival Motor Neuron (Smn) protein levels by modifying Smn exon 7 splicing. Human Molecular Genetics 10, 2727-2736.

  49. Bérubé, N., Jagla, M., Smeenk, C., De Repentigny, Y., Kothary, R., and Picketts, D.  (2002).  Neurodevelopmental defects resulting from ATRX overexpression in Transgenic Mice. Human Molecular Genetics 11, 253-261.

  50. Young, P.J., DiDonato, C.J., Hu, D., Kothary, R., Androphy, E.J., and Lorson, C.L. (2002).  SRp30c-dependent stimulation of survival motor neuron (SMN) exon 7 inclusion is facilitated by a direct interaction with hTra2ß1.  Human Mol. Genetics 11, 577-587.

  51. Marshall, P., Chartrand, N., De Repentigny, Y., Kothary, R., Pelletier, L., Mueller, R., and Worton, R.G. (2002). The mouse dystrophin enhancer preferentially targets lacZ expression in skeletal and cardiac muscle. Developmental Dynamics 224, 30-38.

  52. Campsall, K., Mazarolle, C., De Repentigny, Y., Kothary, R., and Wallace, V. (2002).  Characterization of transgene expression and Cre recombinase activity in a panel of Thy1 promoter-Cre transgenic mice. Developmental Dynamics 224, 135-143.

  53. Saulnier, R., De Repentigny, Y., Yong, V.W., and Kothary, R. (2002). Alterations in myelination in the central nervous system of dystonia musculorum mice. Journal of Neuroscience Research 69, 233-242.

  54. Boudreau-Larivière, C. and Kothary, R. (2002). Differentiation potential of primary myogenic cells derived from skeletal muscle of dystonia musculorum mice.  Differentiation 70:247-256.

  55. DiDonato, C. J., Parks, R. J., and Kothary, R. (2003) Development of a gene therapy strategy for the restoration of survival motor neuron (SMN) expression : implications for SMA therapy. Human Gene Therapy 14, 179-188.

  56. Lee, S. H., Zafer, A., De Repentigny, Y., Kothary, R., Tremblay, M., Gros, P., Duplay, P., Webb, J. R., and Vidal, S. M. (2003) Transgenic expression of the activating natural killer receptor Ly49H confers resistance to cytomegalovirus in genetically susceptible mice.  J. Exp. Medicine. 197, 515-526.

  57. Young, K., Pool, M., and Kothary, R. (2003). Bpag1 localization to actin filaments and to the nucleus is regulated by its N-terminus. Journal of Cell Science, 116, 4543-4555. (Cover figure)

  58. De Repentigny, Y., Deschênes-Furry, J., Jasmin, B.J., and Kothary, R. (2003). Impaired fast axonal transport in neurons of the sciatic nerves from dystonia musculorum mice. Journal of Neurochemistry, 86, 564-571.

  59. De Repentigny, Y., Marshall, P., Worton, R.G., and Kothary, R. (2004). 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, 2853-2862. (Cover figure)

  60. Horsford, D.J., Nguyen, M-T.T., Sellar, G.C., Kothary, R., Arnheiter, H., and McInnes, R.R. (2005). Chx10 repression of Mitf is required for the maintenance of mammalian neuroretinal identity. Development, 132, 177-187.

  61. Young, K., and Kothary, R. (2005). Spectrin repeat proteins in the nucleus. BioEssays, 27, 144-152.

  62. Côté, P.D., De Repentigny, Y., Coupland, S.G., Schwab, Y., Roux, M.J., Levinson, S.R., and Kothary, R. (2005). Physiological maturation of photoreceptors depends on the voltage-gated sodium channel Nav1.6 (Scn8a). J. Neuroscience. 25, 5046-5050. (chosen for the “This week in The Journal” page)

  63. Fernando, P., Ding, W., Pekalska, B., De Repentigny, Y., Kothary, R., Kelly, J.F., and Megeney, L.A. (2005). Activity dependent profiling of protein kinases and kinase substrates using mass spectrometry. Molecular and Cellular Proteomics. 4, 673-682.

  64. Stocksley, M.A., Chakkalakal, J.V., Bradford, A., Miura, P., De Repentigny, Y., Kothary, R., and Jasmin, B.J. (2005). A 1.3 kb Promoter Fragment Confers Spatial and Temporal Expression of Utrophin A mRNA in Mouse Skeletal Muscle Fibers. Neuromuscular Disorders.  15, 437-449.

  65. Shafey, D., Côté, P.D., and Kothary, R. (2005). Characterization of a hypomorphic series of Smn knockdown C2C12 myoblasts reveals intrinsic defects in myoblast fusion and myotube morphology. Experimental Cell Research, 311, 49-61.

  66. Naas, T., Ghorbani, M., Alvarez-Maya, I., Lapner, M., Kothary, R., De Repentigny, Y., Gomes, S., Babiuk, L., Giulivi, A., Soare, C., Azizi, A., and Diaz-Mitoma, F. (2005). Characterization of liver histopathology in a transgenic mouse model expressing genotype 1a Hepatitis C virus core and envelope proteins 1 and 2. J. Gen. Virol. 86, 2185-2196.

  67. Stocksley, M.A., Chakkalakal, J.V., Bradford, A., Miura, P., De Repentigny, Y., Kothary, R., and Jasmin, B.J. (2005). Regulation of utrophin in transgenic mice. Letter in response to Professor K. E. Davies. Neuromuscular Disorders.  15, 648-649.

  68. Pool, M., Boudreau-Larivière, C., Bernier, G., Young, K.G., and Kothary, R. (2005). Genetic alterations at the Bpag1 locus in dt mice and the impact on gene expression. Mammalian Genome, 16, 909-917.

  69. Ichikawa, H., De Repentigny, Y., Kothary, R., and Sugimoto, T. (2006). The survival of vagal and glossopharyngeal sensory neurons is dependent upon dystonin. Neuroscience, 137, 531-536.

  70. Pool, M., McBride, H., Rippstein, P., and Kothary, R. (2006). Trafficking of molecules and organelles in cultured dystonia musculorum sensory neurons is normal. J. Comp. Neurol. 494, 549-558.

  71. Young, K.G., Pinheiro, B., and Kothary, R. (2006). A Bpag1 isoform involved in cytoskeletal organization surrounding the nucleus. Experimental Cell Research, 312, 121-134.

  72. Anderson, C.L., De Repentigny, Y., Cifelli, C., Marshall, P., Renaud, J-M., Worton, R.G., and Kothary, R. (2006). The mouse dystrophin muscle promoter/enhancer is sufficient to drive expression of a mini-dystrophin in transgenic mdx mice and to eliminate the dystrophy in these mice. Molecular Therapy 14, 724-734.

  73. Lee, K., De Repentigny, Y., Saulnier, R., Rippstein, P., Macklin, W., and Kothary, R. (2006). Dominant-negative ?1 integrin mice have region-specific myelin defects accompanied by alterations in MAPK activity. Glia, 53, 836-844.

  74. Karamboulas, C., Dakubo, G.D., Liu, J., De Repentigny, Y., Yutzey, K., Wallace, V.A., Kothary, R., and Skerjanc, I.S. (2006). Disruption of MEF2 activity in cardiomyoblasts inhibits cardiomyogenesis. J. Cell Sci. 119, 4315-4321.

  75. Ichikawa, H., Terayama, R., Yamaai, T., De Repentigny, Y., Kothary, R., and Sugimoto, T. (2007). Dystonin deficiency reduces taste buds and fungiform papillae in the anterior part of the tongue. Brain Research 1129, 142-146.

  76. Young, K.G., De Repentigny, Y., and Kothary, R. (2007). Re: "A possible cellular mechanism of neuronal loss in the dorsal root ganglia of dystonia musculorum (dt) mice". J. Neuropathol. Exp. Neurol. 66, 248-249.

  77. Bowerman, M., Shafey, D., and Kothary, R. (2007). Smn depletion alters Profilin II expression and leads to upregulation of the RhoA/ROCK pathway and defects in neuronal integrity.  J. Mol.  Neurosci. 32, 120-131.

  78. Young, K.G., and Kothary, R. (2007). Dystonin/Bpag1 – a link to what? Cell Motility and the Cytoskeleton. 64, 897-905.

  79. Knight, J.D.R., Qian, B., Baker, D., and Kothary, R. (2007). Conservation, variability and the modeling of active protein kinases. PLoS ONE 2, e982.

  80. Young, K.G., and Kothary, R. (2008). Dystonin/Bpag1 is a necessary endoplasmic reticulum/nuclear envelope protein in sensory neurons. Experimental Cell Research 314, 2750-2761.

  81. Shafey, D., MacKenzie, A., and Kothary, R. (2008). Neurodevelopmental abnormalities in neurosphere-derived neural stem cells from SMN depleted mice. J. Neurosci. Res. 86, 2839-2847.

  82. Ichikawa, H., Terayama, R., Yamaai, T., De Repentigny, Y., Kothary, R., and Sugimoto, T. (2008). 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, 33-38.

  83. Mattis, V., Bowerman, M., Kothary, R., Lorson, C.L. (2008). A SMNDelta7 read-through product confers functionality to the SMNDelta7 protein. Neurosci. Lett. 442, 54-58.

  84. Knight, J.D.R., Hamelberg, D., McCammon, J.A., and Kothary, R. (2009). The role of conserved water molecules in the catalytic domain of protein kinases. Proteins 76, 527-535.

  85. Bowerman, M., Anderson, C.L., Beauvais, A., Boyl, P.P., Witke, W., Kothary, R. (2009). SMN, profilin IIa and plastin 3: a link between the deregulation of actin dynamics and SMA pathogenesis. Mol. Cell. Neurosci. 42, 66-74.

  86. Fernando, P., Sandoz, J.S., Ding, W., De Repentigny, Y., Brunette, S., Kelly, J.F., Kothary, R., and Megeney, L.A. (2009). The Bin1 SH3 domain acts as a scaffold for myofiber sarcomere assembly. J. Biol. Chem. 284, 27674-27686.

  87. Savory, J.G.A., Bouchard, N., Pierre, V., Rijli, F., De Repentigny, Y., Kothary, R., and Lohnes, D. (2009). Cdx2 regulation of posterior development through non-Hox targets. Development. 136, 4099-4110.

  88. Michaud, J-L., Stitt, E., Endlich, N., Endlich, K., De Repentigny, Y., Kothary, R., and Kennedy, C.R.J. (2010). Mice with podocyte-specific overexpression of wild type a-actinin-4 are healthy controls for K256E-a-actinin-4 mutant transgenic mice. Transgenic Res. 19, 285-289.

  89. Liu, H.O., Shafey, D., Moores, J., and Kothary, R. (2010). Neurodevelopmental consequences of Smn depletion in a mouse model of spinal muscular atrophy. J. Neurosci. Res. 88, 111-122.

  90. Miura, P., Coriati, A., Bélanger, G., De Repentigny, Y., Lee, J., Kothary, R., Holcik, M., and Jasmin, B.J. (2010). The utrophin A 5’UTR drives cap-independent translation exclusively in skeletal muscles of transgenic mice and interacts with eEF1A2. Hum. Mol. Gen. 19, 1211-1120.

  91. Bowerman, M., Beauvais, A., Anderson, C.L., and Kothary, R. (2010). Rho-kinase inactivation prolongs survival of an intermediate SMA mouse model. Hum. Mol. Gen. 19, 1468-1478.

  92. *Shafey, D., *Boyer, J.G., Bhanot, K., and Kothary, R. (2010). Identification of novel interacting protein partners of SMN using Tandem Affinity Purification. J. Proteome Res. 9, 1659-1669.

  93. *Boyer, J.G., *Bhanot, K., Kothary, R., and Boudreau-Larivière, C. (2010). Hearts of dystonia musculorum mice display normal morphological and histological features but show signs of cardiac stress. PLOS One 5, e9465.

  94. Poulin, K.L., Lanthier, R.M., Smith, A.C., Christou, C., Quiroz, M.R., Powell, K.L., O'Meara, R.W., Kothary, R., Lorimer, I.A., and Parks, R.J. (2010). Retargeting of adenovirus vectors through genetic fusion of a single-chain or single-domain antibody to capsid protein IX. J. Virol. 84, 10074-10086.

  95. De Repentigny, Y., and Kothary, R. (2010). Production of mouse chimeras by injection of embryonic stem cells into the perivitelline space of one-cell stage embryos. Transgenic Res. 19, 1137-1144. 

  96. Boyer, J.G., Bowerman, M., and Kothary, R. (2010). The many faces of SMN: deciphering the function critical to Spinal Muscular Atrophy Pathogenesis. Future Neurology, 5, 873-890.

  97. Hammond, S.M., Gogliotti, R.G., Rao, V., Beauvais, A., Kothary, R., and DiDonato, C.J. (2010). 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.

  98. Young, K.G., and Kothary, R. (2011). Intermediate filament interactions in neurons. Cytoskeleton of the Nervous System. Editors Ralph A. Nixon and Aidong Yuan. Advances in Neurobiology. 3, 379-410.

  99. *Ferrier, A., *Ryan, S.D., McNally, E.M., and Kothary, R. (2011). New Directions in Biology and Disease of Skeletal Muscle, Meeting Report, 5–8 May 2010, Ottawa, Canada. Neuromuscular Disorders, 21, 157-159.

  100. Liu, H., Beauvais, A., Baker, A.N., Tsilfidis, C., and Kothary, R. (2011). Smn deficiency causes neuritogenesis and neurogenesis defects in the retinal neurons of a mouse model of Spinal Muscular Atrophy. Developmental Neurobiology, 71, 153-169.

  101. O’Meara, R., Michalski, J-P., and Kothary, R. (2011). Integrin signaling in oligodendrocytes and its importance in CNS myelination. J. Signal Transduction, doi:10.1155/2011/354091.

  102. Abdul-Ghani, M., Dufort, D., Stiles, R., De Repentigny, Y., Kothary, R., and Megeney, L. (2011). Wnt11 promotes cardiomyocyte development by caspase-mediated suppression of canonical Wnt signals. Mol. Cell. Biol. 31, 163-178.

  103. *Michalski, J-P., *Anderson, C., Beauvais, A., De Repentigny, Y., and Kothary, R. (2011). The Proteolipid Protein Promoter drives expression outside of the oligodendrocyte lineage during embryonic and early postnatal development. PLOS One, 6, e19772.

  104. Fukuda, K., Knight, J.D.R., Piszczek, G., Kothary, R., and Qin, J. (2011). Biochemical, proteomic, structural, and thermodynamic characterizations of ILK: Cross-validation of the pseudokinase. J. Biol. Chem. 286, 21886-21895.

  105. *De Repentigny, Y., *Ferrier, A., Ryan, S.D., Sato, T., and Kothary, R. (2011). Motor unit abnormalities in dystonia musculorum mice. PLOS One, 6, e21093.

  106. Bowerman, M., Murray, L.M., Beauvais, A., Pinheiro, B., and Kothary, R. (2011) A critical Smn threshold in mice dictates onset of an intermediate Spinal Muscular Atrophy phenotype with a distinct neuromuscular junction pathology.  Neuromuscular Disorders.  In Press.

  107. O’Meara, R.W., Ryan, S.D., Colognato, H., and Kothary, R. (2011). Derivation of enriched oligodendrocyte cultures and oligodendrocyte/neuron myelinating co-cultures from post-natal murine tissues. J Vis Exp. 21, 54.

  108. Bhanot, K., Young, K.G., and Kothary, R. (2011). MAP1B and clathrin are novel interacting partners of the giant cyto-linker dystonin. J. Proteome Res. 10, 5118-27.

  109. Knight, J.D.R., and Kothary, R. (2011). The myogenic kinome: protein kinases critical to mammalian skeletal myogenesis. Skeletal Muscle 1, 29.

  110. Poulin, K.L., Tong, G., Vorobyova, O., Pool, M., Kothary, R., and Parks, R.J. (2011). Cre/loxP recombimnation can be used to swap cell binding motifs on the adenoviral capsid protein IX. Virology.  Virology 420, 146-155.

  111. Knight, JDR., Tian, R., Lee, REC., Wang, F., Zou, H., Megeney, LA., Figeys, D., and Kothary, R. (2011). A novel whole-cell lysate kinase assay identifies substrates of the p38 MAPK in differentiating myoblasts. Skeletal Muscle Manuscript submitted.

  112. *Ryan, S.D., *Ferrier, A., Sato, T., O’Meara, R.W., De Repentigny, Y., Jiang, S.X., Hou, S.T., and Kothary, R. (2011). Neuronal dystonin isoform 2 is a novel mediator of endoplasmic reticulum structure and function. Mol. Biol. Cell Manuscript under revision.

  113. Ryan, S.D., Bhanot, K., De Repentigny, Y., Chu, A., Blais, A., and Kothary, R. (2011). Microtubule stability, Golgi organization, and transport flux require dystonin-a2/MAP1B interaction. J. Cell Biol. Manuscript under revision.

  114. Slack, R., Ghanem, N., Andrusiak, M., Svoboda, D., Julian, L., McClellan, K., De Repentigny, Y., Kothary, R., Ekker, M., Blais, A., Park, D.  (2011) The RB/E2F Pathway modulates Neurogenesis Through Direct Regulation of DLX2.  The EMBO Journal.  Manuscript Submitted.