Published in J Cell Biol on September 01, 2003
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Spinal muscular atrophy: why do low levels of survival motor neuron protein make motor neurons sick? Nat Rev Neurosci (2009) 4.16
Smn, the spinal muscular atrophy-determining gene product, modulates axon growth and localization of beta-actin mRNA in growth cones of motoneurons. J Cell Biol (2003) 3.40
Antisense correction of SMN2 splicing in the CNS rescues necrosis in a type III SMA mouse model. Genes Dev (2010) 3.02
Reduced SMN protein impairs maturation of the neuromuscular junctions in mouse models of spinal muscular atrophy. Hum Mol Genet (2008) 2.82
Ribonucleoprotein assembly defects correlate with spinal muscular atrophy severity and preferentially affect a subset of spliceosomal snRNPs. PLoS One (2007) 2.70
EPHA4 is a disease modifier of amyotrophic lateral sclerosis in animal models and in humans. Nat Med (2012) 2.65
Plastin 3 is a protective modifier of autosomal recessive spinal muscular atrophy. Science (2008) 2.51
Neuronal SMN expression corrects spinal muscular atrophy in severe SMA mice while muscle-specific SMN expression has no phenotypic effect. Hum Mol Genet (2008) 2.35
Reduced U snRNP assembly causes motor axon degeneration in an animal model for spinal muscular atrophy. Genes Dev (2005) 2.22
A single administration of morpholino antisense oligomer rescues spinal muscular atrophy in mouse. Hum Mol Genet (2011) 2.13
Abnormal motor phenotype in the SMNDelta7 mouse model of spinal muscular atrophy. Neurobiol Dis (2007) 2.10
The motor neuron response to SMN1 deficiency in spinal muscular atrophy. Muscle Nerve (2014) 2.02
An SMN-dependent U12 splicing event essential for motor circuit function. Cell (2012) 1.94
Multiprotein complexes of the survival of motor neuron protein SMN with Gemins traffic to neuronal processes and growth cones of motor neurons. J Neurosci (2006) 1.80
Embryonic motor axon development in the severe SMA mouse. Hum Mol Genet (2008) 1.79
Modeling spinal muscular atrophy in Drosophila. PLoS One (2008) 1.71
Therapeutics development for spinal muscular atrophy. NeuroRx (2006) 1.62
Severe neuromuscular denervation of clinically relevant muscles in a mouse model of spinal muscular atrophy. Hum Mol Genet (2011) 1.61
A Drosophila melanogaster model of spinal muscular atrophy reveals a function for SMN in striated muscle. J Cell Biol (2007) 1.60
Effects of 2,4-diaminoquinazoline derivatives on SMN expression and phenotype in a mouse model for spinal muscular atrophy. Hum Mol Genet (2009) 1.46
Temporal requirement for high SMN expression in SMA mice. Hum Mol Genet (2011) 1.44
Motor neuron mitochondrial dysfunction in spinal muscular atrophy. Hum Mol Genet (2016) 1.41
A genetic model of amyotrophic lateral sclerosis in zebrafish displays phenotypic hallmarks of motoneuron disease. Dis Model Mech (2010) 1.36
Interaction of survival of motor neuron (SMN) and HuD proteins with mRNA cpg15 rescues motor neuron axonal deficits. Proc Natl Acad Sci U S A (2011) 1.32
A SMN missense mutation complements SMN2 restoring snRNPs and rescuing SMA mice. Hum Mol Genet (2009) 1.31
Spinal muscular atrophy: the role of SMN in axonal mRNA regulation. Brain Res (2012) 1.26
Zebrafish survival motor neuron mutants exhibit presynaptic neuromuscular junction defects. Hum Mol Genet (2009) 1.24
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Dysregulation of ubiquitin homeostasis and β-catenin signaling promote spinal muscular atrophy. J Clin Invest (2014) 1.08
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
SMN complex localizes to the sarcomeric Z-disc and is a proteolytic target of calpain. Hum Mol Genet (2008) 1.07
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Proteomic assessment of a cell model of spinal muscular atrophy. BMC Neurosci (2011) 1.05
RNA-Seq analysis in mutant zebrafish reveals role of U1C protein in alternative splicing regulation. EMBO J (2011) 1.05
Limited phenotypic effects of selectively augmenting the SMN protein in the neurons of a mouse model of severe spinal muscular atrophy. PLoS One (2012) 1.03
A screen for regulators of survival of motor neuron protein levels. Nat Chem Biol (2011) 1.02
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A Drosophila model of spinal muscular atrophy uncouples snRNP biogenesis functions of survival motor neuron from locomotion and viability defects. Cell Rep (2012) 1.01
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A cell-autonomous defect in skeletal muscle satellite cells expressing low levels of survival of motor neuron protein. Dev Biol (2012) 1.00
RNA processing defects associated with diseases of the motor neuron. Muscle Nerve (2010) 0.99
Axonal-SMN (a-SMN), a protein isoform of the survival motor neuron gene, is specifically involved in axonogenesis. Proc Natl Acad Sci U S A (2007) 0.99
ZPR1 is essential for survival and is required for localization of the survival motor neurons (SMN) protein to Cajal bodies. Mol Cell Biol (2005) 0.99
Absence of gemin5 from SMN complexes in nuclear Cajal bodies. BMC Cell Biol (2007) 0.98
The zinc finger protein ZPR1 is a potential modifier of spinal muscular atrophy. Hum Mol Genet (2012) 0.98
SMN requirement for synaptic vesicle, active zone and microtubule postnatal organization in motor nerve terminals. PLoS One (2011) 0.97
Temporal requirement for SMN in motoneuron development. Hum Mol Genet (2013) 0.96
Modeling spinal muscular atrophy in Drosophila links Smn to FGF signaling. J Cell Biol (2011) 0.96
Antisense oligonucleotides for the treatment of spinal muscular atrophy. Hum Gene Ther (2013) 0.95
RNA-binding proteins and translational regulation in axons and growth cones. Front Neurosci (2013) 0.95
Calcium binding is essential for plastin 3 function in Smn-deficient motoneurons. Hum Mol Genet (2013) 0.94
Generation and Characterization of a genetic zebrafish model of SMA carrying the human SMN2 gene. Mol Neurodegener (2011) 0.94
Specific splicing defects in S. pombe carrying a degron allele of the Survival of Motor Neuron gene. EMBO J (2010) 0.93
Gemin3 is an essential gene required for larval motor function and pupation in Drosophila. Mol Biol Cell (2008) 0.92
Involvement of Rho-family GTPases in axon branching. Small GTPases (2014) 0.91
Reduced levels of survival motor neuron protein leads to aberrant motoneuron growth in a Xenopus model of muscular atrophy. Neurogenetics (2009) 0.91
Targeting RNA-splicing for SMA treatment. Mol Cells (2012) 0.91
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A large animal model of spinal muscular atrophy and correction of phenotype. Ann Neurol (2015) 0.90
The neuroprotective factor Wld(s) fails to mitigate distal axonal and neuromuscular junction (NMJ) defects in mouse models of spinal muscular atrophy. Neurosci Lett (2008) 0.89
Therapy development in spinal muscular atrophy. Nat Neurosci (2010) 0.88
Dilysine motifs in exon 2b of SMN protein mediate binding to the COPI vesicle protein α-COP and neurite outgrowth in a cell culture model of spinal muscular atrophy. Hum Mol Genet (2013) 0.88
The E3 ubiquitin ligase mind bomb 1 ubiquitinates and promotes the degradation of survival of motor neuron protein. Mol Biol Cell (2013) 0.88
Neuromuscular effects of G93A-SOD1 expression in zebrafish. Mol Neurodegener (2012) 0.88
Knockdown of Pnpla6 protein results in motor neuron defects in zebrafish. Dis Model Mech (2012) 0.87
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Deficiency of the Survival of Motor Neuron Protein Impairs mRNA Localization and Local Translation in the Growth Cone of Motor Neurons. J Neurosci (2016) 0.83
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
Increased susceptibility of spinal muscular atrophy fibroblasts to camptothecin is p53-independent. BMC Cell Biol (2009) 0.83
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Abnormal interaction of motor neuropathy-associated mutant HspB8 (Hsp22) forms with the RNA helicase Ddx20 (gemin3). Cell Stress Chaperones (2010) 0.83
Copy Number Variations in the Survival Motor Neuron Genes: Implications for Spinal Muscular Atrophy and Other Neurodegenerative Diseases. Front Mol Biosci (2016) 0.83
The Met receptor tyrosine kinase prevents zebrafish primary motoneurons from expressing an incorrect neurotransmitter. Neural Dev (2008) 0.82
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The SMN protein is a key regulator of nuclear architecture in differentiating neuroblastoma cells. Traffic (2009) 0.82
Diverse role of Survival Motor Neuron Protein. Biochim Biophys Acta (2017) 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
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The Gemin associates of survival motor neuron are required for motor function in Drosophila. PLoS One (2013) 0.81
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Reduced SMN protein impairs maturation of the neuromuscular junctions in mouse models of spinal muscular atrophy. Hum Mol Genet (2008) 2.82
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Effects of 2,4-diaminoquinazoline derivatives on SMN expression and phenotype in a mouse model for spinal muscular atrophy. Hum Mol Genet (2009) 1.46
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Interaction of survival of motor neuron (SMN) and HuD proteins with mRNA cpg15 rescues motor neuron axonal deficits. Proc Natl Acad Sci U S A (2011) 1.32
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A SMN missense mutation complements SMN2 restoring snRNPs and rescuing SMA mice. Hum Mol Genet (2009) 1.31
A role for complexes of survival of motor neurons (SMN) protein with gemins and profilin in neurite-like cytoplasmic extensions of cultured nerve cells. Exp Cell Res (2005) 1.29
Zebrafish survival motor neuron mutants exhibit presynaptic neuromuscular junction defects. Hum Mol Genet (2009) 1.24
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A novel method for oral delivery of drug compounds to the neonatal SMNDelta7 mouse model of spinal muscular atrophy. J Neurosci Methods (2006) 1.00
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Calcium binding is essential for plastin 3 function in Smn-deficient motoneurons. Hum Mol Genet (2013) 0.94
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