Research Topics
Genomes and Genes
Species | Rashmi KotharySummaryAffiliation: Ottawa Health Research Institute Country: Canada Publications
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Detail Information
Publications
A novel whole-cell lysate kinase assay identifies substrates of the p38 MAPK in differentiating myoblastsJames Dr Knight
Regenerative Medicine Program, Ottawa Hospital Research Institute, 501 Smyth Road, Ottawa, ON, K1H 8L6, Canada
Skelet Muscle 2:5. 2012..abstract:..- Fasudil improves survival and promotes skeletal muscle development in a mouse model of spinal muscular atrophyMelissa Bowerman
Ottawa Hospital Research Institute, 501 Smyth Road, Ottawa, ON, Canada K1H 8L6
BMC Med 10:24. 2012..In the present study, we evaluated the therapeutic potential of the clinically approved ROCK inhibitor fasudil... - The myogenic kinome: protein kinases critical to mammalian skeletal myogenesisJames Dr Knight
Regenerative Medicine Program, Ottawa Hospital Research Institute, 501 Smyth Road, Ottawa, ON, K1H 8L6, Canada
Skelet Muscle 1:29. 2011..We present a scheme of protein kinase activity, similar to that which exists for the myogenic transcription factors, to better clarify the complex signalling that underlies muscle development... - Neuronal dystonin isoform 2 is a mediator of endoplasmic reticulum structure and functionScott D Ryan
Ottawa Hospital Research Institute, Ottawa, ON K1H 8L6, Canada
Mol Biol Cell 23:553-66. 2012..This study provides insight into the mechanism of dt neuropathology and proposes a role for dystonin-a2 as a mediator of normal ER structure and function... - Microtubule stability, Golgi organization, and transport flux require dystonin-a2-MAP1B interactionScott D Ryan
Ottawa Hospital Research Institute, Ottawa, Ontario K1H 8L6, Canada
J Cell Biol 196:727-42. 2012..These cellular aberrations are apparent in prephenotype dorsal root ganglia and primary sensory neurons from dt mice, suggesting they are causal in the disorder... 
Rho-kinase inactivation prolongs survival of an intermediate SMA mouse modelMelissa Bowerman
Ottawa Hospital Research Institute, Ottawa, ON, Canada K1H 8L6
Hum Mol Genet 19:1468-78. 2010..Our study presents evidence linking disruption of actin cytoskeletal dynamics to SMA pathogenesis and, for the first time, identifies RhoA effectors as viable targets for therapeutic intervention in the disease...- A critical smn threshold in mice dictates onset of an intermediate spinal muscular atrophy phenotype associated with a distinct neuromuscular junction pathologyMelissa Bowerman
Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, ON, Canada K1H 8L6
Neuromuscul Disord 22:263-76. 2012..Further, the finding that NMJ pathology varies between severe and intermediate SMA mouse models, suggests that future therapies be adapted to the severity of SMA... - Neurodevelopmental consequences of Smn depletion in a mouse model of spinal muscular atrophyHong Liu
Ottawa Hospital Research Institute and The University of Ottawa Center for Neuromuscular Disease, Ottawa, Ontario, Canada
J Neurosci Res 88:111-22. 2010..Overall, we conclude that Smn depletion affects developmental processes, which ultimately may also contribute to SMA pathogenesis... - MAP1B and clathrin are novel interacting partners of the giant cyto-linker dystoninKunal Bhanot
Ottawa Hospital Research Institute, Ottawa, Ontario, Canada
J Proteome Res 10:5118-27. 2011..This work will further facilitate our understanding of how cytoskeletal proteins can affect and regulate neurodegenerative disorders... 
The mouse dystrophin muscle enhancer-1 imparts skeletal muscle, but not cardiac muscle, expression onto the dystrophin Purkinje promoter in transgenic miceYves De Repentigny
Ottawa Health Research Institute, University of Ottawa Center for Neuromuscular Disease, Ottawa, Ontario, Canada
Hum Mol Genet 13:2853-62. 2004..Furthermore, they provide support for the model in which muscle enhancers, like DME-1, activate the dystrophin B and CP promoters in skeletal muscle, but not in cardiac muscle, of XLCM patients...- Motor unit abnormalities in Dystonia musculorum miceYves De Repentigny
Ottawa Hospital Research Institute, Ottawa, Ontario, Canada
PLoS ONE 6:e21093. 2011..This study reveals neuromuscular defects that likely contribute to the dt(27J) pathology and identifies a critical role for dystonin outside of sensory neurons... - Smn deficiency causes neuritogenesis and neurogenesis defects in the retinal neurons of a mouse model of spinal muscular atrophyHong Liu
Ottawa Hospital Research Institute, Ottawa, Ontario, Canada K1H 8L6
Dev Neurobiol 71:153-69. 2011.... - Hypomorphic Smn knockdown C2C12 myoblasts reveal intrinsic defects in myoblast fusion and myotube morphologyDina Shafey
Ottawa Health Research Institute, Ottawa, ON, Canada
Exp Cell Res 311:49-61. 2005..Taken together, our work supports the view that there is an intrinsic defect in skeletal muscle cells of SMA patients and that this defect contributes to the overall pathogenesis in this devastating disease... - Mice with podocyte-specific overexpression of wild type alpha-actinin-4 are healthy controls for K256E-alpha-actinin-4 mutant transgenic miceJean Louis Michaud
Kidney Research Centre, Division of Nephrology, Department of Medicine, Ottawa Health Research Institute, Ottawa Hospital and University of Ottawa, Ottawa, ON K1H 8M5, Canada
Transgenic Res 19:285-9. 2010..These findings suggest that the K256E mutation itself and not overexpression of alpha-actinin-4 protein per se accounts for the FSGS phenotype in our transgenic model... - Identification of novel interacting protein partners of SMN using tandem affinity purificationDina Shafey
Ottawa Hospital Research Institute, Ottawa, ON, Canada
J Proteome Res 9:1659-69. 2010..The discovery of these proteins will lead to a better understanding of the mechanisms underlying the pathophysiology of SMA... - Mouse dystrophin enhancer preferentially targets lacZ expression in skeletal and cardiac musclePhilip Marshall
Ottawa Health Research Institute, Ottawa, Ontario, K1H 8L6, Canada
Dev Dyn 224:30-8. 2002..Our results also suggest that sequences surrounding the mouse dystrophin enhancer may affect its function throughout mouse development... - Development of a gene therapy strategy for the restoration of survival motor neuron protein expression: implications for spinal muscular atrophy therapyChristine J DiDonato
Ottawa Health Research Institute, Molecular Medicine Program and University of Ottawa Center for Neuromuscular Disease, Ottawa, ON, K1N 8L6, Canada
Hum Gene Ther 14:179-88. 2003.... - SMN, profilin IIa and plastin 3: a link between the deregulation of actin dynamics and SMA pathogenesisMelissa Bowerman
Ottawa Hospital Research Institute, Ottawa, ON, Canada
Mol Cell Neurosci 42:66-74. 2009..However, the depletion of profilin II and the restoration of plastin 3 are not sufficient to rescue the SMA phenotype. Our study suggests that additional regulators of actin dynamics must also contribute to SMA pathogenesis... - Neurodevelopmental abnormalities in neurosphere-derived neural stem cells from SMN-depleted miceDina Shafey
Ottawa Health Research Institute, Ottawa, Ontario, Canada
J Neurosci Res 86:2839-47. 2008..Our work raises the possibility that SMN depletion affects neurodevelopment and neuromaintenance to varying extents, leading to SMA pathogenesis... - Wnt11 promotes cardiomyocyte development by caspase-mediated suppression of canonical Wnt signalsMohammad Abdul-Ghani
Ottawa Hospital Research Institute, Regenerative Medicine Program, Ottawa Hospital, General Campus, Ottawa, Ontario K1H 8L6, Canada
Mol Cell Biol 31:163-78. 2011..Taken together, these results suggest that noncanonical Wnt signals promote myocyte maturation through caspase-mediated inhibition of β-catenin activity... - The mouse dystrophin muscle promoter/enhancer drives expression of mini-dystrophin in transgenic mdx mice and rescues the dystrophy in these miceCarrie L Anderson
Ottawa Health Research Institute, Ottawa, ON, Canada K1H 8L6
Mol Ther 14:724-34. 2006..Therefore, the dystrophin muscle promoter/enhancer sequence represents an alternative for use in gene therapy vectors for the treatment of DMD... - Retargeting of adenovirus vectors through genetic fusion of a single-chain or single-domain antibody to capsid protein IXKathy L Poulin
Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, Ontario, Canada
J Virol 84:10074-86. 2010..Taken together, our data indicate that pIX is an effective platform for presentation of large targeting polypeptides on the surface of the virus capsid, but the nature of the ligand can significantly affect its association with virions... - Cdx2 regulation of posterior development through non-Hox targetsJoanne G A Savory
Department of Cellular and Molecular Medicine, University of Ottawa, Ottawa, Ontario, K1H 8M5, Canada
Development 136:4099-110. 2009..We propose a model wherein Cdx2 functions as an integrator of caudalizing information by coordinating axial elongation and somite patterning through Hox-independent and -dependent pathways, respectively... - Bin1 SRC homology 3 domain acts as a scaffold for myofiber sarcomere assemblyPasan Fernando
The Sprott Center for Stem Cell Research, Regenerative Medicine Program, Ottawa, Ontario K1H 8L6, Canada
J Biol Chem 284:27674-86. 2009..Collectively, these observations suggest that Bin1 displays protein scaffold-like properties and binds with sarcomeric factors important in directing sarcomere protein assembly and myofiber maturation... - The Rb/E2F pathway modulates neurogenesis through direct regulation of the Dlx1/Dlx2 bigene clusterNoel Ghanem
Department of Cellular and Molecular Medicine, University of Ottawa, Ottawa, Ontario K1H 8M5, Canada
J Neurosci 32:8219-30. 2012.... - The proteolipid protein promoter drives expression outside of the oligodendrocyte lineage during embryonic and early postnatal developmentJohn Paul Michalski
Ottawa Hospital Research Institute, Ottawa, Ontario, Canada
PLoS ONE 6:e19772. 2011..Understanding the temporal shift in Plp driven expression is of consequence when designing experimental models to study oligodendrocyte biology... - Smn depletion alters profilin II expression and leads to upregulation of the RhoA/ROCK pathway and defects in neuronal integrityMelissa Bowerman
Ottawa Health Research Institute, Ottawa, ON, Canada
J Mol Neurosci 32:120-31. 2007..This study represents the first description of a mechanism underlying SMA pathogenesis and highlights new targets for therapeutic intervention for this devastating disorder... - Genetic alterations at the Bpag1 locus in dt mice and their impact on transcript expressionMadeline Pool
Molecular Medicine Program, Ottawa Health Research Institute, Ottawa, Ontario, K1H 8L6, Canada
Mamm Genome 16:909-17. 2005..Such alterations likely result in reduced or absent dystonin/Bpag1 protein levels. Thus, distinct genetic defects lead to a common outcome of reduced transcript expression causing the same phenotype in multiple dt alleles... - Integrin signaling in oligodendrocytes and its importance in CNS myelinationRyan W O'Meara
Regenerative Medicine Program, Ottawa Hospital Research Institute, 501 Smyth Road, Ottawa, ON, Canada K1H 8L6
J Signal Transduct 2011:354091. 2011..Here, we review the current understanding of this important signaling axis and its role in oligodendrocyte biology and ultimately in the myelination of axons within the CNS... - Production of mouse chimeras by injection of embryonic stem cells into the perivitelline space of one-cell stage embryosYves De Repentigny
Ottawa Hospital Research Institute, 501 Smyth Road, Ottawa, ON, K1H 8L6, Canada
Transgenic Res 19:1137-44. 2010..This method allows for the earlier introduction of ES cells into mouse embryos, and should free up the possibility of using frozen one-cell embryos for this purpose... - Impaired fast axonal transport in neurons of the sciatic nerves from dystonia musculorum miceYves De Repentigny
Ottawa Health Research Institute, Ottawa, Ontario, Canada
J Neurochem 86:564-71. 2003..Our findings demonstrate that axonal transport of AChE in both orthograde and retrograde directions is markedly affected, and allow us to conclude that axonal transport defects do exist in the sciatic nerves of dt27J mice... - Dystonin/Bpag1 is a necessary endoplasmic reticulum/nuclear envelope protein in sensory neuronsKevin G Young
Ottawa Health Research Institute, 501 Smyth Road, Ottawa, Ontario, Canada K1H 8L6
Exp Cell Res 314:2750-61. 2008..Defects in dt mice, as demonstrated here, may ultimately result in pathogenesis involving ER dysfunction and contribute significantly to the dt phenotype... - Characterization of transgene expression and Cre recombinase activity in a panel of Thy-1 promoter-Cre transgenic miceKatrina D Campsall
Molecular Medicine Program, Center for Neuromuscular Disease, Ottawa Health Research Institute, Ottawa, ON, Canada
Dev Dyn 224:135-43. 2002..This panel of Thy1-Cre transgenic mice, however, will be useful reagents for the ablation of genes whose transcripts are spatially or temporally restricted in the developing NS... - A Bpag1 isoform involved in cytoskeletal organization surrounding the nucleusKevin G Young
Ottawa Health Research Institute, 501 Smyth Road, Ottawa, Ontario, Canada K1H 8L6
Exp Cell Res 312:121-34. 2006..The overall structure and subcellular localizations of Bpag1a2 indicate possible functions in nuclear envelope structuring, nuclear tethering, and organization of membranous structures surrounding the nucleus... - Alterations in myelination in the central nervous system of dystonia musculorum miceRon Saulnier
Ottawa Health Research Institute and The University of Ottawa Center for Neuromuscular Disease, Ottawa, Ontario, Canada
J Neurosci Res 69:233-42. 2002..Short-term cultures of oligodendrocytes derived from dt(Tg4) mice did not show morphological alterations... - Bpag1 localization to actin filaments and to the nucleus is regulated by its N-terminusKevin G Young
Ottawa Health Research Institute, 501 Smyth Road, Ottawa, Ontario, Canada K1H 8L6, and Center for Neuromuscular Disease, and Departments of Cellular and Molecular Medicine, and Medicine, University of Ottawa, Ottawa, Ontario, Canada K1H 8M5
J Cell Sci 116:4543-55. 2003..We provide the first indication that Bpag1 is not strictly a cytoplasmic/membrane protein but that it can also localize to the nucleus... - Transgenic expression of the activating natural killer receptor Ly49H confers resistance to cytomegalovirus in genetically susceptible miceSeung Hwan Lee
Department of Biochemistry, Microbiology and Immunology, University of Ottawa, Ottawa, Ontario K1H 8M5, Canada
J Exp Med 197:515-26. 2003..This panel of transgenic animals provides a unique resource to study possible pleiotropic effect of Cmv1... - Neurodevelopmental defects resulting from ATRX overexpression in transgenic miceNathalie G Berube
Ottawa Health Research Institute, Ottawa, Ontario, Canada
Hum Mol Genet 11:253-61. 2002..Our findings indicate that ATRX dosage is crucial for normal development and organization of the cortex, and emphasize the relevance of our model for the study of ATRX function and disease pathogenesis... - Active kinase proteome screening reveals novel signal complexity in cardiomyopathyPasan Fernando
Molecular Medicine Program, Ottawa Health Research Institute, Ottawa Hospital, Ottawa, Ontario K1H 8L6, Canada
Mol Cell Proteomics 4:673-82. 2005.... - Physiological maturation of photoreceptors depends on the voltage-gated sodium channel NaV1.6 (Scn8a)Patrice D Côté
Molecular Medicine Program, Ottawa Health Research Institute, and University of Ottawa Center for Neuromuscular Disease, Ottawa, Ontario, K1H 8L6, Canada
J Neurosci 25:5046-50. 2005..Together, our data reveal a previously unappreciated role for VGSCs in the physiological maturation of photoreceptors... - Characterization of liver histopathology in a transgenic mouse model expressing genotype 1a hepatitis C virus core and envelope proteins 1 and 2Turaya Naas
Division of Virology, Children's Hospital of Eastern Ontario, 401 Smyth Road, Ottawa, ON, Canada, K1H 8L1
J Gen Virol 86:2185-96. 2005..It is likely that the HCV structural proteins mediate some of the histological alterations in hepatocytes by interfering with lipid transport and liver metabolism... - Trafficking of macromolecules and organelles in cultured Dystonia musculorum sensory neurons is normalMadeline Pool
Molecular Medicine Program, Ottawa Health Research Institute, Ottawa, Ontario K1H 8L6, Canada
J Comp Neurol 494:549-58. 2006..In addition, dystonin/Bpag1 is not an essential part of the dynein motor complex for mitochondrial transport since mitochondrial trafficking is normal in cultured sensory neurons from dt mice... - Dominant-negative beta1 integrin mice have region-specific myelin defects accompanied by alterations in MAPK activityKaren K Lee
Ottawa Health Research Institute, Ottawa, ON, Canada K1H 8L6
Glia 53:836-44. 2006..Our work highlights the importance of beta1 integrin-mediated signaling in CNS myelination in vivo...