Rashmi Kothary

Regenerative Medicine Program, Ottawa Hospital Research Institute

Affiliated withOttawa Hospital Research Institute University of Ottawa

Research Area

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JoVE Journal Publications

ArticleTotal : 2	Year
Publication title Cited by 106	2011
Publication title Cited by 22	2014

Other Publications

Article	Year
Neurodevelopmental defects resulting from ATRX overexpression in transgenic mice. Human molecular genetics\| PubMed ID: 11823444	2002
SRp30c-dependent stimulation of survival motor neuron (SMN) exon 7 inclusion is facilitated by a direct interaction with hTra2 beta 1. Human molecular genetics\| PubMed ID: 11875052	2002
Mouse dystrophin enhancer preferentially targets lacZ expression in skeletal and cardiac muscle. Developmental dynamics : an official publication of the American Association of Anatomists\| PubMed ID: 11984871	2002
Alterations in myelination in the central nervous system of dystonia musculorum mice. Journal of neuroscience research\| PubMed ID: 12111805	2002
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\| PubMed ID: 12112467	2002
Differentiation potential of primary myogenic cells derived from skeletal muscle of dystonia musculorum mice. Differentiation; research in biological diversity\| PubMed ID: 12190986	2002
Transgenic expression of the activating natural killer receptor Ly49H confers resistance to cytomegalovirus in genetically susceptible mice. The Journal of experimental medicine\| PubMed ID: 12591908	2003
Development of a gene therapy strategy for the restoration of survival motor neuron protein expression: implications for spinal muscular atrophy therapy. Human gene therapy\| PubMed ID: 12614569	2003
Impaired fast axonal transport in neurons of the sciatic nerves from dystonia musculorum mice. Journal of neurochemistry\| PubMed ID: 12859670	2003
Bpag1 localization to actin filaments and to the nucleus is regulated by its N-terminus. Journal of cell science\| PubMed ID: 14576348	2003
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\| PubMed ID: 15385445	2004
Chx10 repression of Mitf is required for the maintenance of mammalian neuroretinal identity. Development (Cambridge, England)\| PubMed ID: 15576400	2005
Spectrin repeat proteins in the nucleus. BioEssays : news and reviews in molecular, cellular and developmental biology\| PubMed ID: 15666356	2005
Active kinase proteome screening reveals novel signal complexity in cardiomyopathy. Molecular & cellular proteomics : MCP\| PubMed ID: 15722372	2005
Physiological maturation of photoreceptors depends on the voltage-gated sodium channel NaV1.6 (Scn8a). The Journal of neuroscience : the official journal of the Society for Neuroscience\| PubMed ID: 15901786	2005
A 1.3 kb promoter fragment confers spatial and temporal expression of utrophin A mRNA in mouse skeletal muscle fibers. Neuromuscular disorders : NMD\| PubMed ID: 15907291	2005
Characterization of liver histopathology in a transgenic mouse model expressing genotype 1a hepatitis C virus core and envelope proteins 1 and 2. The Journal of general virology\| PubMed ID: 16033966	2005
Hypomorphic Smn knockdown C2C12 myoblasts reveal intrinsic defects in myoblast fusion and myotube morphology. Experimental cell research\| PubMed ID: 16219305	2005
A Bpag1 isoform involved in cytoskeletal organization surrounding the nucleus. Experimental cell research\| PubMed ID: 16289082	2006
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\| PubMed ID: 16341670	2005
Trafficking of macromolecules and organelles in cultured Dystonia musculorum sensory neurons is normal. The Journal of comparative neurology\| PubMed ID: 16374799	2006
Dominant-negative beta1 integrin mice have region-specific myelin defects accompanied by alterations in MAPK activity. Glia\| PubMed ID: 16575886	2006
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\| PubMed ID: 16807118	2006
Disruption of MEF2 activity in cardiomyoblasts inhibits cardiomyogenesis. Journal of cell science\| PubMed ID: 17003108	2006
Re: "A possible cellular mechanism of neuronal loss in the dorsal root ganglia of dystonia musculorum (dt) mice". Journal of neuropathology and experimental neurology\| PubMed ID: 17356386	2007
Dystonin/Bpag1--a link to what? Cell motility and the cytoskeleton\| PubMed ID: 17849487	2007
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\| PubMed ID: 17873296	2007
Conservation, variability and the modeling of active protein kinases. PloS one\| PubMed ID: 17912359	2007
Neurodevelopmental abnormalities in neurosphere-derived neural stem cells from SMN-depleted mice. Journal of neuroscience research\| PubMed ID: 18521935	2008
A SMNDelta7 read-through product confers functionality to the SMNDelta7 protein. Neuroscience letters\| PubMed ID: 18601974	2008
Dystonin/Bpag1 is a necessary endoplasmic reticulum/nuclear envelope protein in sensory neurons. Experimental cell research\| PubMed ID: 18638474	2008
The role of conserved water molecules in the catalytic domain of protein kinases. Proteins\| PubMed ID: 19425109	2009
SMN, profilin IIa and plastin 3: a link between the deregulation of actin dynamics and SMA pathogenesis. Molecular and cellular neurosciences\| PubMed ID: 19497369	2009
Mice with podocyte-specific overexpression of wild type alpha-actinin-4 are healthy controls for K256E-alpha-actinin-4 mutant transgenic mice. Transgenic research\| PubMed ID: 19585264	2010
Bin1 SRC homology 3 domain acts as a scaffold for myofiber sarcomere assembly. The Journal of biological chemistry\| PubMed ID: 19633357	2009
Neurodevelopmental consequences of Smn depletion in a mouse model of spinal muscular atrophy. Journal of neuroscience research\| PubMed ID: 19642194	2010
Cdx2 regulation of posterior development through non-Hox targets. Development (Cambridge, England)\| PubMed ID: 19906845	2009
Rho-kinase inactivation prolongs survival of an intermediate SMA mouse model. Human molecular genetics\| PubMed ID: 20097679	2010
Production of mouse chimeras by injection of embryonic stem cells into the perivitelline space of one-cell stage embryos. Transgenic research\| PubMed ID: 20143261	2010
Identification of novel interacting protein partners of SMN using tandem affinity purification. Journal of proteome research\| PubMed ID: 20201562	2010
Hearts of dystonia musculorum mice display normal morphological and histological features but show signs of cardiac stress. PloS one\| PubMed ID: 20209123	2010
Retargeting of adenovirus vectors through genetic fusion of a single-chain or single-domain antibody to capsid protein IX. Journal of virology\| PubMed ID: 20631131	2010
Smn deficiency causes neuritogenesis and neurogenesis defects in the retinal neurons of a mouse model of spinal muscular atrophy. Developmental neurobiology\| PubMed ID: 20862739	2011
Wnt11 promotes cardiomyocyte development by caspase-mediated suppression of canonical Wnt signals. Molecular and cellular biology\| PubMed ID: 21041481	2011
Mouse survival motor neuron alleles that mimic SMN2 splicing and are inducible rescue embryonic lethality early in development but not late. PloS one\| PubMed ID: 21249120	2010
Biochemical, proteomic, structural, and thermodynamic characterizations of integrin-linked kinase (ILK): cross-validation of the pseudokinase. The Journal of biological chemistry\| PubMed ID: 21524996	2011
The proteolipid protein promoter drives expression outside of the oligodendrocyte lineage during embryonic and early postnatal development. PloS one\| PubMed ID: 21572962	2011
Integrin signaling in oligodendrocytes and its importance in CNS myelination. Journal of signal transduction\| PubMed ID: 21637375	2011
Motor unit abnormalities in Dystonia musculorum mice. PloS one\| PubMed ID: 21698255	2011
The myogenic kinome: protein kinases critical to mammalian skeletal myogenesis. Skeletal muscle\| PubMed ID: 21902831	2011
MAP1B and clathrin are novel interacting partners of the giant cyto-linker dystonin. Journal of proteome research\| PubMed ID: 21936565	2011
Use of Cre/loxP recombination to swap cell binding motifs on the adenoviral capsid protein IX. Virology\| PubMed ID: 21968200	2011
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\| PubMed ID: 22071333	2011
Neuronal dystonin isoform 2 is a mediator of endoplasmic reticulum structure and function. Molecular biology of the cell\| PubMed ID: 22190742	2012
A novel whole-cell lysate kinase assay identifies substrates of the p38 MAPK in differentiating myoblasts. Skeletal muscle\| PubMed ID: 22394512	2012
Fasudil improves survival and promotes skeletal muscle development in a mouse model of spinal muscular atrophy. BMC medicine\| PubMed ID: 22397316	2012
Microtubule stability, Golgi organization, and transport flux require dystonin-a2-MAP1B interaction. The Journal of cell biology\| PubMed ID: 22412020	2012
Untethering the nuclear envelope and cytoskeleton: biologically distinct dystonias arising from a common cellular dysfunction. International journal of cell biology\| PubMed ID: 22611399	2012
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\| PubMed ID: 22699903	2012
A novel role for the cytoskeletal linker protein dystonin in the maintenance of microtubule stability and the regulation of ER-Golgi transport. Bioarchitecture\| PubMed ID: 22754620	2012
Glucose metabolism and pancreatic defects in spinal muscular atrophy. Annals of neurology\| PubMed ID: 22926856	2012
Defects in neuromuscular junction remodelling in the Smn(2B/-) mouse model of spinal muscular atrophy. Neurobiology of disease\| PubMed ID: 22960106	2012
A novel function for the survival motoneuron protein as a translational regulator. Human molecular genetics\| PubMed ID: 23136128	2013
Cellular and molecular biology of neuronal dystonin. International review of cell and molecular biology\| PubMed ID: 23273860	2013
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\| PubMed ID: 23739974	2013
Distinct roles for Ste20-like kinase SLK in muscle function and regeneration. Skeletal muscle\| PubMed ID: 23815977	2013
Six1 regulates MyoD expression in adult muscle progenitor cells. PloS one\| PubMed ID: 23840772	2013
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\| PubMed ID: 23859020	2013
Early onset muscle weakness and disruption of muscle proteins in mouse models of spinal muscular atrophy. Skeletal muscle\| PubMed ID: 24119341	2013
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\| PubMed ID: 24381311	2013
More than a bystander: the contributions of intrinsic skeletal muscle defects in motor neuron diseases. Frontiers in physiology\| PubMed ID: 24391590	2013