Rashmi Kothary

Summary

Affiliation: Ottawa Health Research Institute
Country: Canada

Publications

  1. pmc The proteolipid protein promoter drives expression outside of the oligodendrocyte lineage during embryonic and early postnatal development
    John Paul Michalski
    Ottawa Hospital Research Institute, Ottawa, Ontario, Canada
    PLoS ONE 6:e19772. 2011
  2. pmc Microtubule stability, Golgi organization, and transport flux require dystonin-a2-MAP1B interaction
    Scott D Ryan
    Ottawa Hospital Research Institute, Ottawa, Ontario K1H 8L6, Canada
    J Cell Biol 196:727-42. 2012
  3. pmc Motor unit abnormalities in Dystonia musculorum mice
    Yves De Repentigny
    Ottawa Hospital Research Institute, Ottawa, Ontario, Canada
    PLoS ONE 6:e21093. 2011
  4. pmc Integrin signaling in oligodendrocytes and its importance in CNS myelination
    Ryan W O'Meara
    Regenerative Medicine Program, Ottawa Hospital Research Institute, 501 Smyth Road, Ottawa, ON, Canada K1H 8L6
    J Signal Transduct 2011:354091. 2011
  5. pmc Conservation, variability and the modeling of active protein kinases
    James D R Knight
    Molecular Medicine Program, Ottawa Health Research Institute, Ottawa, Ontario, Canada
    PLoS ONE 2:e982. 2007
  6. pmc A novel whole-cell lysate kinase assay identifies substrates of the p38 MAPK in differentiating myoblasts
    James Dr Knight
    Regenerative Medicine Program, Ottawa Hospital Research Institute, 501 Smyth Road, Ottawa, ON, K1H 8L6, Canada
    Skelet Muscle 2:5. 2012
  7. pmc The myogenic kinome: protein kinases critical to mammalian skeletal myogenesis
    James Dr Knight
    Regenerative Medicine Program, Ottawa Hospital Research Institute, 501 Smyth Road, Ottawa, ON, K1H 8L6, Canada
    Skelet Muscle 1:29. 2011
  8. pmc Neuronal dystonin isoform 2 is a mediator of endoplasmic reticulum structure and function
    Scott D Ryan
    Ottawa Hospital Research Institute, Ottawa, ON K1H 8L6, Canada
    Mol Biol Cell 23:553-66. 2012
  9. ncbi request reprint Bpag1 localization to actin filaments and to the nucleus is regulated by its N-terminus
    Kevin 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
  10. doi request reprint Dystonin/Bpag1 is a necessary endoplasmic reticulum/nuclear envelope protein in sensory neurons
    Kevin G Young
    Ottawa Health Research Institute, 501 Smyth Road, Ottawa, Ontario, Canada K1H 8L6
    Exp Cell Res 314:2750-61. 2008

Collaborators

Detail Information

Publications63

  1. pmc The proteolipid protein promoter drives expression outside of the oligodendrocyte lineage during embryonic and early postnatal development
    John 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...
  2. pmc Microtubule stability, Golgi organization, and transport flux require dystonin-a2-MAP1B interaction
    Scott 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...
  3. pmc Motor unit abnormalities in Dystonia musculorum mice
    Yves 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...
  4. pmc Integrin signaling in oligodendrocytes and its importance in CNS myelination
    Ryan 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...
  5. pmc Conservation, variability and the modeling of active protein kinases
    James D R Knight
    Molecular Medicine Program, Ottawa Health Research Institute, Ottawa, Ontario, Canada
    PLoS ONE 2:e982. 2007
    ....
  6. pmc A novel whole-cell lysate kinase assay identifies substrates of the p38 MAPK in differentiating myoblasts
    James Dr Knight
    Regenerative Medicine Program, Ottawa Hospital Research Institute, 501 Smyth Road, Ottawa, ON, K1H 8L6, Canada
    Skelet Muscle 2:5. 2012
    ..abstract:..
  7. pmc The myogenic kinome: protein kinases critical to mammalian skeletal myogenesis
    James 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...
  8. pmc Neuronal dystonin isoform 2 is a mediator of endoplasmic reticulum structure and function
    Scott 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...
  9. ncbi request reprint Bpag1 localization to actin filaments and to the nucleus is regulated by its N-terminus
    Kevin 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...
  10. doi request reprint Dystonin/Bpag1 is a necessary endoplasmic reticulum/nuclear envelope protein in sensory neurons
    Kevin 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...
  11. ncbi request reprint A Bpag1 isoform involved in cytoskeletal organization surrounding the nucleus
    Kevin 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...
  12. pmc Hearts of dystonia musculorum mice display normal morphological and histological features but show signs of cardiac stress
    Justin G Boyer
    Department of Biology, Laurentian University, Sudbury, Ontario, Canada
    PLoS ONE 5:e9465. 2010
    ....
  13. doi request reprint MAP1B and clathrin are novel interacting partners of the giant cyto-linker dystonin
    Kunal 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...
  14. ncbi request reprint SMN, profilin IIa and plastin 3: a link between the deregulation of actin dynamics and SMA pathogenesis
    Melissa 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...
  15. doi request reprint A critical smn threshold in mice dictates onset of an intermediate spinal muscular atrophy phenotype associated with a distinct neuromuscular junction pathology
    Melissa 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...
  16. ncbi request reprint Dominant-negative beta1 integrin mice have region-specific myelin defects accompanied by alterations in MAPK activity
    Karen 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...
  17. pmc Six1 regulates MyoD expression in adult muscle progenitor cells
    Yubing Liu
    Ottawa Institute of Systems Biology, University of Ottawa, Ottawa, Ontario, Canada
    PLoS ONE 8:e67762. 2013
    ..Together, our results reveal that MyoD expression in satellite cells depends on Six1, supporting the idea that Six1 plays an important role in adult myogenesis, in addition to its role in embryonic muscle formation. ..
  18. doi request reprint Neurodevelopmental consequences of Smn depletion in a mouse model of spinal muscular atrophy
    Hong 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...
  19. doi request reprint Rho-kinase inactivation prolongs survival of an intermediate SMA mouse model
    Melissa 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...
  20. doi request reprint Smn deficiency causes neuritogenesis and neurogenesis defects in the retinal neurons of a mouse model of spinal muscular atrophy
    Hong Liu
    Ottawa Hospital Research Institute, Ottawa, Ontario, Canada K1H 8L6
    Dev Neurobiol 71:153-69. 2011
    ....
  21. ncbi request reprint Differentiation potential of primary myogenic cells derived from skeletal muscle of dystonia musculorum mice
    Céline Boudreau-Larivière
    Ottawa Health Research Institute, 501 Smyth Road, Ontario, Canada K1H 8L6
    Differentiation 70:247-56. 2002
    ..Together, these findings demonstrate that the early phases of myogenic differentiation occur independently of Bpag1...
  22. ncbi request reprint Genetic alterations at the Bpag1 locus in dt mice and their impact on transcript expression
    Madeline 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...
  23. pmc Fasudil improves survival and promotes skeletal muscle development in a mouse model of spinal muscular atrophy
    Melissa 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...
  24. ncbi request reprint Trafficking of macromolecules and organelles in cultured Dystonia musculorum sensory neurons is normal
    Madeline 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...
  25. doi request reprint Integrin-linked kinase regulates process extension in oligodendrocytes via control of actin cytoskeletal dynamics
    Ryan W O'Meara
    Ottawa Hospital Research Institute, Ottawa, Ontario K1H 8L6, Canada
    J Neurosci 33:9781-93. 2013
    ....
  26. ncbi request reprint Hypomorphic Smn knockdown C2C12 myoblasts reveal intrinsic defects in myoblast fusion and myotube morphology
    Dina 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...
  27. ncbi request reprint Disruption of MEF2 activity in cardiomyoblasts inhibits cardiomyogenesis
    Christina Karamboulas
    Department of Biochemistry, Medical Sciences Building, University of Western Ontario, London, Ontario, N6A 5C1, Canada
    J Cell Sci 119:4315-21. 2006
    ..Our results show that MEF2C, or genes containing MEF2 DNA-binding sites, is required for the efficient differentiation of cardiomyoblasts into cardiomyocytes, suggesting conservation in the role of MEF2 from Drosophila to mammals...
  28. ncbi request reprint Alterations in myelination in the central nervous system of dystonia musculorum mice
    Ron 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...
  29. ncbi request reprint Smn depletion alters profilin II expression and leads to upregulation of the RhoA/ROCK pathway and defects in neuronal integrity
    Melissa 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...
  30. doi request reprint Supraphysiological expression of survival motor neuron protein from an adenovirus vector does not adversely affect cell function
    Benoit B Goulet
    Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, ON, Canada
    Biochem Cell Biol 91:252-64. 2013
    ..However, normal human fibroblasts treated with Ad-SMN showed a slight reduction in growth rate, suggesting that certain cell types may be differently impacted by high levels of SMN. ..
  31. pmc Retargeting of adenovirus vectors through genetic fusion of a single-chain or single-domain antibody to capsid protein IX
    Kathy 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...
  32. ncbi request reprint Impaired fast axonal transport in neurons of the sciatic nerves from dystonia musculorum mice
    Yves 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...
  33. ncbi request reprint The mouse dystrophin muscle promoter/enhancer drives expression of mini-dystrophin in transgenic mdx mice and rescues the dystrophy in these mice
    Carrie 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...
  34. pmc Bin1 SRC homology 3 domain acts as a scaffold for myofiber sarcomere assembly
    Pasan 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...
  35. doi request reprint Neurodevelopmental abnormalities in neurosphere-derived neural stem cells from SMN-depleted mice
    Dina 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...
  36. doi request reprint Use of Cre/loxP recombination to swap cell binding motifs on the adenoviral capsid protein IX
    Kathy L Poulin
    Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, Ontario, Canada
    Virology 420:146-55. 2011
    ..Thus, we have developed a simple method to propagate virus lacking native viral tropism but containing cell-specific binding ligands...
  37. doi request reprint Identification of novel interacting protein partners of SMN using tandem affinity purification
    Dina 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...
  38. doi request reprint Cellular and molecular biology of neuronal dystonin
    Andrew Ferrier
    Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, Ontario, Canada
    Int Rev Cell Mol Biol 300:85-120. 2013
    ..This chapter provides a comprehensive overview of the dystonin gene, the structure of encoded proteins, biological functions of neuronal dystonin isoforms, and known roles of dystonin in dt pathogenesis and human disease...
  39. ncbi request reprint Dystonin/Bpag1--a link to what?
    Kevin G Young
    Ottawa Health Research Institute, Ottawa, Ontario, K1H 8L6 Canada
    Cell Motil Cytoskeleton 64:897-905. 2007
    ..Elucidating the roles of dystonin most relevant to neuronal function and survival should help to shed light on some of the common mechanisms underlying neurodegeneration...
  40. pmc Transgenic expression of neuronal dystonin isoform 2 partially rescues the disease phenotype of the dystonia musculorum mouse model of hereditary sensory autonomic neuropathy VI
    Andrew Ferrier
    Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, Ontario, Canada K1H 8L6
    Hum Mol Genet 23:2694-710. 2014
    ..Taken together, this study provides needed insight into the molecular basis of the dt disorder and other peripheral neuropathies including HSAN-VI. ..
  41. doi request reprint The Rb/E2F pathway modulates neurogenesis through direct regulation of the Dlx1/Dlx2 bigene cluster
    Noel Ghanem
    Department of Cellular and Molecular Medicine, University of Ottawa, Ottawa, Ontario K1H 8M5, Canada
    J Neurosci 32:8219-30. 2012
    ....
  42. ncbi request reprint The mouse dystrophin muscle enhancer-1 imparts skeletal muscle, but not cardiac muscle, expression onto the dystrophin Purkinje promoter in transgenic mice
    Yves 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...
  43. ncbi request reprint Characterization of transgene expression and Cre recombinase activity in a panel of Thy-1 promoter-Cre transgenic mice
    Katrina 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...
  44. doi request reprint Cdx2 regulation of posterior development through non-Hox targets
    Joanne 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...
  45. doi request reprint Mice with podocyte-specific overexpression of wild type alpha-actinin-4 are healthy controls for K256E-alpha-actinin-4 mutant transgenic mice
    Jean 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...
  46. pmc Defects in pancreatic development and glucose metabolism in SMN-depleted mice independent of canonical spinal muscular atrophy neuromuscular pathology
    Melissa Bowerman
    Ottawa Hospital Research Institute, Ottawa, Ontario, Canada The Neuroscience Institute of Montpellier INM, INSERM UMR1051, Saint Eloi Hospital, Montpellier, France
    Hum Mol Genet 23:3432-44. 2014
    ....
  47. ncbi request reprint 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...
  48. ncbi request reprint Spectrin repeat proteins in the nucleus
    Kevin G Young
    Ottawa Health Research Institute, Ontario K1H 8L6, Canada
    Bioessays 27:144-52. 2005
    ..Supplementary material for this article can be found on the BioEssays website (http://www.interscience.wiley.com/jpages/0265-9247/suppmat/index.html)...
  49. ncbi request reprint Glucose metabolism and pancreatic defects in spinal muscular atrophy
    Melissa Bowerman
    Ottawa Hospital Research Institute, Ottawa, Canada
    Ann Neurol 72:256-68. 2012
    ..We thus initiated an in-depth analysis of glucose metabolism in SMA...
  50. ncbi request reprint Characterization of liver histopathology in a transgenic mouse model expressing genotype 1a hepatitis C virus core and envelope proteins 1 and 2
    Turaya 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...
  51. ncbi request reprint Mouse dystrophin enhancer preferentially targets lacZ expression in skeletal and cardiac muscle
    Philip 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...
  52. pmc Transgenic expression of the activating natural killer receptor Ly49H confers resistance to cytomegalovirus in genetically susceptible mice
    Seung 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...
  53. pmc The smn-independent beneficial effects of trichostatin a on an intermediate mouse model of spinal muscular atrophy
    Hong Liu
    Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, Ontario, Canada
    PLoS ONE 9:e101225. 2014
    ..As such, identification of the pathways regulated by TSA in the Smn2B/- mice could lead to the development of novel therapeutics for treating SMA. ..
  54. ncbi request reprint Active kinase proteome screening reveals novel signal complexity in cardiomyopathy
    Pasan Fernando
    Molecular Medicine Program, Ottawa Health Research Institute, Ottawa Hospital, Ottawa, Ontario K1H 8L6, Canada
    Mol Cell Proteomics 4:673-82. 2005
    ....
  55. pmc The role of conserved water molecules in the catalytic domain of protein kinases
    James D R Knight
    Ottawa Hospital Research Institute, Ontario, Canada
    Proteins 76:527-35. 2009
    ..Our results highlight the importance of novel elements within the greater kinase family and suggest that conserved water molecules are necessary for efficient kinase function...
  56. pmc Wnt11 promotes cardiomyocyte development by caspase-mediated suppression of canonical Wnt signals
    Mohammad 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...
  57. doi request reprint A novel function for the survival motoneuron protein as a translational regulator
    Gabriel Sanchez
    Department of Cellular and Molecular Medicine, University of Ottawa, Ottawa, ON, Canada K1H 8M5
    Hum Mol Genet 22:668-84. 2013
    ..Importantly, an SMA-causing mutation in the Tudor domain of SMN completely abolished translational repression, a strong indication for the functional significance of this novel SMN activity in the pathology...
  58. ncbi request reprint Development of a gene therapy strategy for the restoration of survival motor neuron protein expression: implications for spinal muscular atrophy therapy
    Christine 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
    ....
  59. doi request reprint Production of mouse chimeras by injection of embryonic stem cells into the perivitelline space of one-cell stage embryos
    Yves 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...
  60. ncbi request reprint Neurodevelopmental defects resulting from ATRX overexpression in transgenic mice
    Nathalie 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...