Published in Hum Mol Genet on January 22, 2010
Neurotrophic requirements of human motor neurons defined using amplified and purified stem cell-derived cultures. PLoS One (2014) 1.45
Spinal muscular atrophy: the role of SMN in axonal mRNA regulation. Brain Res (2012) 1.26
Spinal muscular atrophy: a motor neuron disorder or a multi-organ disease? J Anat (2013) 1.23
Glucose metabolism and pancreatic defects in spinal muscular atrophy. Ann Neurol (2012) 1.17
Assays for the identification and prioritization of drug candidates for spinal muscular atrophy. Assay Drug Dev Technol (2014) 1.13
The COPI vesicle complex binds and moves with survival motor neuron within axons. Hum Mol Genet (2011) 1.08
Survival motor neuron affects plastin 3 protein levels leading to motor defects. J Neurosci (2012) 1.07
Fasudil improves survival and promotes skeletal muscle development in a mouse model of spinal muscular atrophy. BMC Med (2012) 1.05
ROCKing Regeneration: Rho Kinase Inhibition as Molecular Target for Neurorestoration. Front Mol Neurosci (2011) 1.02
Spinal muscular atrophy: development and implementation of potential treatments. Ann Neurol (2013) 1.01
Axon growth inhibition by RhoA/ROCK in the central nervous system. Front Neurosci (2014) 0.97
Mouse models of SMA: tools for disease characterization and therapeutic development. Hum Genet (2012) 0.96
Calcium binding is essential for plastin 3 function in Smn-deficient motoneurons. Hum Mol Genet (2013) 0.94
Early onset muscle weakness and disruption of muscle proteins in mouse models of spinal muscular atrophy. Skelet Muscle (2013) 0.94
Prolactin increases SMN expression and survival in a mouse model of severe spinal muscular atrophy via the STAT5 pathway. J Clin Invest (2011) 0.94
Disease mechanisms and therapeutic approaches in spinal muscular atrophy. J Neurosci (2015) 0.92
SMN-inducing compounds for the treatment of spinal muscular atrophy. Future Med Chem (2012) 0.90
Myogenic program dysregulation is contributory to disease pathogenesis in spinal muscular atrophy. Hum Mol Genet (2014) 0.90
Advances in therapeutic development for spinal muscular atrophy. Future Med Chem (2014) 0.89
A humanized Smn gene containing the SMN2 nucleotide alteration in exon 7 mimics SMN2 splicing and the SMA disease phenotype. Hum Mol Genet (2010) 0.89
Rac1 at the crossroad of actin dynamics and neuroinflammation in Amyotrophic Lateral Sclerosis. Front Cell Neurosci (2014) 0.88
Genetic circuitry of Survival motor neuron, the gene underlying spinal muscular atrophy. Proc Natl Acad Sci U S A (2013) 0.86
Enhancement of SMN protein levels in a mouse model of spinal muscular atrophy using novel drug-like compounds. EMBO Mol Med (2013) 0.84
Dysfunction of axonal membrane conductances in adolescents and young adults with spinal muscular atrophy. Brain (2011) 0.84
Defects in pancreatic development and glucose metabolism in SMN-depleted mice independent of canonical spinal muscular atrophy neuromuscular pathology. Hum Mol Genet (2014) 0.83
RhoA mediates defective stem cell function and heterotopic ossification in dystrophic muscle of mice. FASEB J (2013) 0.83
Pharmacological inhibition of Rho-kinase (ROCK) signaling enhances cisplatin resistance in neuroblastoma cells. Int J Oncol (2010) 0.82
Spinal muscular atrophy: from tissue specificity to therapeutic strategies. F1000Prime Rep (2015) 0.82
Diverse role of Survival Motor Neuron Protein. Biochim Biophys Acta (2017) 0.81
The rho kinase inhibitor Y-27632 improves motor performance in male SOD1(G93A) mice. Front Neurosci (2014) 0.81
Analysis of the fibroblast growth factor system reveals alterations in a mouse model of spinal muscular atrophy. PLoS One (2012) 0.81
Chatting with the neighbors: crosstalk between Rho-kinase (ROCK) and other signaling pathways for treatment of neurological disorders. Front Neurosci (2015) 0.81
Genome-wide analysis shows association of epigenetic changes in regulators of Rab and Rho GTPases with spinal muscular atrophy severity. Eur J Hum Genet (2013) 0.81
Emerging therapies and challenges in Spinal Muscular Atrophy. Ann Neurol (2016) 0.80
A cell system for phenotypic screening of modifiers of SMN2 gene expression and function. PLoS One (2013) 0.80
Neurogenic and myogenic contributions to hereditary motor neuron disease. Neurodegener Dis (2012) 0.80
Molecular Mechanisms of Neurodegeneration in Spinal Muscular Atrophy. J Exp Neurosci (2016) 0.80
Genetic inhibition of JNK3 ameliorates spinal muscular atrophy. Hum Mol Genet (2015) 0.80
The Smn-independent beneficial effects of trichostatin A on an intermediate mouse model of spinal muscular atrophy. PLoS One (2014) 0.79
Is spinal muscular atrophy a disease of the motor neurons only: pathogenesis and therapeutic implications? Cell Mol Life Sci (2015) 0.78
Protective effects of butyrate-based compounds on a mouse model for spinal muscular atrophy. Exp Neurol (2016) 0.78
Therapy development for spinal muscular atrophy in SMN independent targets. Neural Plast (2012) 0.78
ROCK inhibition as a therapy for spinal muscular atrophy: understanding the repercussions on multiple cellular targets. Front Neurosci (2014) 0.78
Effect of genetic background on the phenotype of the Smn2B/- mouse model of spinal muscular atrophy. Hum Mol Genet (2016) 0.76
Long-term exercise-specific neuroprotection in spinal muscular atrophy-like mice. J Physiol (2016) 0.76
Small molecule suppressors of Drosophila kinesin deficiency rescue motor axon development in a zebrafish model of spinal muscular atrophy. PLoS One (2013) 0.76
The effect of the DcpS inhibitor D156844 on the protective action of follistatin in mice with spinal muscular atrophy. Neuromuscul Disord (2015) 0.76
Rho Kinase Inhibition with Fasudil in the SOD1(G93A) Mouse Model of Amyotrophic Lateral Sclerosis-Symptomatic Treatment Potential after Disease Onset. Front Pharmacol (2017) 0.75
Established Stem Cell Model of Spinal Muscular Atrophy Is Applicable in the Evaluation of the Efficacy of Thyrotropin-Releasing Hormone Analog. Stem Cells Transl Med (2015) 0.75
Therapeutic strategies for spinal muscular atrophy: SMN and beyond. Dis Model Mech (2017) 0.75
A SAGE approach to discovery of genes involved in autophagic cell death. Curr Biol (2003) 2.40
Chx10 repression of Mitf is required for the maintenance of mammalian neuroretinal identity. Development (2004) 1.79
SRp30c-dependent stimulation of survival motor neuron (SMN) exon 7 inclusion is facilitated by a direct interaction with hTra2 beta 1. Hum Mol Genet (2002) 1.53
A 1.3 kb promoter fragment confers spatial and temporal expression of utrophin A mRNA in mouse skeletal muscle fibers. Neuromuscul Disord (2005) 1.45
Transgenic expression of the activating natural killer receptor Ly49H confers resistance to cytomegalovirus in genetically susceptible mice. J Exp Med (2003) 1.37
IAPs regulate the plasticity of cell migration by directly targeting Rac1 for degradation. EMBO J (2011) 1.36
SMN, profilin IIa and plastin 3: a link between the deregulation of actin dynamics and SMA pathogenesis. Mol Cell Neurosci (2009) 1.26
Hypomorphic Smn knockdown C2C12 myoblasts reveal intrinsic defects in myoblast fusion and myotube morphology. Exp Cell Res (2005) 1.23
A critical smn threshold in mice dictates onset of an intermediate spinal muscular atrophy phenotype associated with a distinct neuromuscular junction pathology. Neuromuscul Disord (2011) 1.20
Glucose metabolism and pancreatic defects in spinal muscular atrophy. Ann Neurol (2012) 1.17
Structure of the capsular polysaccharide of pneumococcal serotype 11A reveals a novel acetylglycerol that is the structural basis for 11A subtypes. J Biol Chem (2008) 1.15
Neurodevelopmental defects resulting from ATRX overexpression in transgenic mice. Hum Mol Genet (2002) 1.13
Smn depletion alters profilin II expression and leads to upregulation of the RhoA/ROCK pathway and defects in neuronal integrity. J Mol Neurosci (2007) 1.12
Mouse survival motor neuron alleles that mimic SMN2 splicing and are inducible rescue embryonic lethality early in development but not late. PLoS One (2010) 1.11
Derivation of enriched oligodendrocyte cultures and oligodendrocyte/neuron myelinating co-cultures from post-natal murine tissues. J Vis Exp (2011) 1.10
The myogenic kinome: protein kinases critical to mammalian skeletal myogenesis. Skelet Muscle (2011) 1.10
Fasudil improves survival and promotes skeletal muscle development in a mouse model of spinal muscular atrophy. BMC Med (2012) 1.05
Dominant-negative beta1 integrin mice have region-specific myelin defects accompanied by alterations in MAPK activity. Glia (2006) 1.04
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 (2005) 1.04
Microtubule stability, Golgi organization, and transport flux require dystonin-a2-MAP1B interaction. J Cell Biol (2012) 1.03
Wnt11 promotes cardiomyocyte development by caspase-mediated suppression of canonical Wnt signals. Mol Cell Biol (2010) 1.02
Disruption of MEF2 activity in cardiomyoblasts inhibits cardiomyogenesis. J Cell Sci (2006) 0.99
Biochemical, proteomic, structural, and thermodynamic characterizations of integrin-linked kinase (ILK): cross-validation of the pseudokinase. J Biol Chem (2011) 0.99
Dystonin/Bpag1--a link to what? Cell Motil Cytoskeleton (2007) 0.99
Dystonin/Bpag1 is a necessary endoplasmic reticulum/nuclear envelope protein in sensory neurons. Exp Cell Res (2008) 0.97
A SMNDelta7 read-through product confers functionality to the SMNDelta7 protein. Neurosci Lett (2008) 0.97
Cdx2 regulation of posterior development through non-Hox targets. Development (2009) 0.96
Conservation, variability and the modeling of active protein kinases. PLoS One (2007) 0.95
A novel function for the survival motoneuron protein as a translational regulator. Hum Mol Genet (2012) 0.95
Spectrin repeat proteins in the nucleus. Bioessays (2005) 0.94
A Bpag1 isoform involved in cytoskeletal organization surrounding the nucleus. Exp Cell Res (2005) 0.94
Early onset muscle weakness and disruption of muscle proteins in mouse models of spinal muscular atrophy. Skelet Muscle (2013) 0.94
Genetic alterations at the Bpag1 locus in dt mice and their impact on transcript expression. Mamm Genome (2005) 0.94
The Rb/E2F pathway modulates neurogenesis through direct regulation of the Dlx1/Dlx2 bigene cluster. J Neurosci (2012) 0.93
Characterization of transgene expression and Cre recombinase activity in a panel of Thy-1 promoter-Cre transgenic mice. Dev Dyn (2002) 0.93
Bin1 SRC homology 3 domain acts as a scaffold for myofiber sarcomere assembly. J Biol Chem (2009) 0.90
Bpag1 localization to actin filaments and to the nucleus is regulated by its N-terminus. J Cell Sci (2003) 0.88
Neurodevelopmental consequences of Smn depletion in a mouse model of spinal muscular atrophy. J Neurosci Res (2010) 0.88
Identification of novel interacting protein partners of SMN using tandem affinity purification. J Proteome Res (2010) 0.88
Neuronal dystonin isoform 2 is a mediator of endoplasmic reticulum structure and function. Mol Biol Cell (2011) 0.88
Smn deficiency causes neuritogenesis and neurogenesis defects in the retinal neurons of a mouse model of spinal muscular atrophy. Dev Neurobiol (2011) 0.88
Retargeting of adenovirus vectors through genetic fusion of a single-chain or single-domain antibody to capsid protein IX. J Virol (2010) 0.88
A novel whole-cell lysate kinase assay identifies substrates of the p38 MAPK in differentiating myoblasts. Skelet Muscle (2012) 0.88
Neurodevelopmental abnormalities in neurosphere-derived neural stem cells from SMN-depleted mice. J Neurosci Res (2008) 0.87
Integrin signaling in oligodendrocytes and its importance in CNS myelination. J Signal Transduct (2010) 0.87
Untethering the nuclear envelope and cytoskeleton: biologically distinct dystonias arising from a common cellular dysfunction. Int J Cell Biol (2012) 0.87
Six1 regulates MyoD expression in adult muscle progenitor cells. PLoS One (2013) 0.87
Physiological maturation of photoreceptors depends on the voltage-gated sodium channel NaV1.6 (Scn8a). J Neurosci (2005) 0.86
The proteolipid protein promoter drives expression outside of the oligodendrocyte lineage during embryonic and early postnatal development. PLoS One (2011) 0.86
MAP1B and clathrin are novel interacting partners of the giant cyto-linker dystonin. J Proteome Res (2011) 0.85
Motor unit abnormalities in Dystonia musculorum mice. PLoS One (2011) 0.84
Integrin-linked kinase regulates process extension in oligodendrocytes via control of actin cytoskeletal dynamics. J Neurosci (2013) 0.84
Dissection of the transversus abdominis muscle for whole-mount neuromuscular junction analysis. J Vis Exp (2014) 0.84
The role of conserved water molecules in the catalytic domain of protein kinases. Proteins (2009) 0.83
Development of a gene therapy strategy for the restoration of survival motor neuron protein expression: implications for spinal muscular atrophy therapy. Hum Gene Ther (2003) 0.82
Hearts of dystonia musculorum mice display normal morphological and histological features but show signs of cardiac stress. PLoS One (2010) 0.82
A novel role for the cytoskeletal linker protein dystonin in the maintenance of microtubule stability and the regulation of ER-Golgi transport. Bioarchitecture (2012) 0.81
Cellular and molecular biology of neuronal dystonin. Int Rev Cell Mol Biol (2013) 0.81
Impaired fast axonal transport in neurons of the sciatic nerves from dystonia musculorum mice. J Neurochem (2003) 0.81
Production of mouse chimeras by injection of embryonic stem cells into the perivitelline space of one-cell stage embryos. Transgenic Res (2010) 0.80
Disruption in the autophagic process underlies the sensory neuropathy in dystonia musculorum mice. Autophagy (2015) 0.79
The mouse dystrophin muscle promoter/enhancer drives expression of mini-dystrophin in transgenic mdx mice and rescues the dystrophy in these mice. Mol Ther (2006) 0.79
Biochemical and cellular analysis of human variants of the DYT1 dystonia protein, TorsinA/TOR1A. Hum Mutat (2014) 0.79
Trafficking of macromolecules and organelles in cultured Dystonia musculorum sensory neurons is normal. J Comp Neurol (2006) 0.79
Mice with podocyte-specific overexpression of wild type alpha-actinin-4 are healthy controls for K256E-alpha-actinin-4 mutant transgenic mice. Transgenic Res (2009) 0.79
Differentiation potential of primary myogenic cells derived from skeletal muscle of dystonia musculorum mice. Differentiation (2002) 0.78
Supraphysiological expression of survival motor neuron protein from an adenovirus vector does not adversely affect cell function. Biochem Cell Biol (2013) 0.78
Use of Cre/loxP recombination to swap cell binding motifs on the adenoviral capsid protein IX. Virology (2011) 0.78
Identification of 3-O-acetylglycerol, a novel structural element in bacterial polysaccharides. Carbohydr Res (2009) 0.78
Transgenic expression of neuronal dystonin isoform 2 partially rescues the disease phenotype of the dystonia musculorum mouse model of hereditary sensory autonomic neuropathy VI. Hum Mol Genet (2013) 0.78
The mouse dystrophin muscle enhancer-1 imparts skeletal muscle, but not cardiac muscle, expression onto the dystrophin Purkinje promoter in transgenic mice. Hum Mol Genet (2004) 0.78
Active kinase proteome screening reveals novel signal complexity in cardiomyopathy. Mol Cell Proteomics (2005) 0.77
Alterations in myelination in the central nervous system of dystonia musculorum mice. J Neurosci Res (2002) 0.77
Distinct roles for Ste20-like kinase SLK in muscle function and regeneration. Skelet Muscle (2013) 0.76
Re: "A possible cellular mechanism of neuronal loss in the dorsal root ganglia of dystonia musculorum (dt) mice". J Neuropathol Exp Neurol (2007) 0.76
Corrigendum: Establishment of a cone photoreceptor transplantation platform based on a novel cone-GFP reporter mouse line. Sci Rep (2016) 0.75
Mouse dystrophin enhancer preferentially targets lacZ expression in skeletal and cardiac muscle. Dev Dyn (2002) 0.75
Structural elucidation of an α-1,2-mannosidase resistant oligosaccharide produced in Pichia pastoris. Glycobiology (2011) 0.75
Use of imaged capillary isoelectric focusing technique in development of diphtheria toxin mutant CRM197. Electrophoresis (2014) 0.75