Published in PLoS One on September 26, 2007
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SMN deficiency causes tissue-specific perturbations in the repertoire of snRNAs and widespread defects in splicing. Cell (2008) 4.54
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The DcpS inhibitor RG3039 improves motor function in SMA mice. Hum Mol Genet (2013) 0.99
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The zinc finger protein ZPR1 is a potential modifier of spinal muscular atrophy. Hum Mol Genet (2012) 0.98
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Spliceosomal small nuclear ribonucleoprotein biogenesis defects and motor neuron selectivity in spinal muscular atrophy. Brain Res (2012) 0.91
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The role of nuclear bodies in gene expression and disease. Biology (Basel) (2013) 0.91
SMN is essential for the biogenesis of U7 small nuclear ribonucleoprotein and 3'-end formation of histone mRNAs. Cell Rep (2013) 0.91
SMN-inducing compounds for the treatment of spinal muscular atrophy. Future Med Chem (2012) 0.90
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Ubiquitin-specific protease 9x deubiquitinates and stabilizes the spinal muscular atrophy protein-survival motor neuron. J Biol Chem (2012) 0.88
Spinal muscular atrophy: journeying from bench to bedside. Neurotherapeutics (2014) 0.87
Solution structure of the core SMN-Gemin2 complex. Biochem J (2012) 0.86
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The spinal muscular atrophy mouse model, SMAΔ7, displays altered axonal transport without global neurofilament alterations. Acta Neuropathol (2011) 0.86
Splicing changes in SMA mouse motoneurons and SMN-depleted neuroblastoma cells: evidence for involvement of splicing regulatory proteins. RNA Biol (2014) 0.84
A U1 snRNP-specific assembly pathway reveals the SMN complex as a versatile hub for RNP exchange. Nat Struct Mol Biol (2016) 0.83
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The SMN protein is a key regulator of nuclear architecture in differentiating neuroblastoma cells. Traffic (2009) 0.82
A role for the Cajal-body-associated SUMO isopeptidase USPL1 in snRNA transcription mediated by RNA polymerase II. J Cell Sci (2014) 0.82
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Genome-wide identification of mRNAs associated with the protein SMN whose depletion decreases their axonal localization. RNA (2013) 0.82
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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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RNA-sequencing of a mouse-model of spinal muscular atrophy reveals tissue-wide changes in splicing of U12-dependent introns. Nucleic Acids Res (2016) 0.80
Implication of the SMN complex in the biogenesis and steady state level of the signal recognition particle. Nucleic Acids Res (2012) 0.80
Neurogenic and myogenic contributions to hereditary motor neuron disease. Neurodegener Dis (2012) 0.80
A cell system for phenotypic screening of modifiers of SMN2 gene expression and function. PLoS One (2013) 0.80
Neurofilament Phosphorylation during Development and Disease: Which Came First, the Phosphorylation or the Accumulation? J Amino Acids (2012) 0.79
Behavioral and electrophysiological outcomes of tissue-specific Smn knockdown in Drosophila melanogaster. Brain Res (2012) 0.79
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A single nucleotide in the SMN gene regulates splicing and is responsible for spinal muscular atrophy. Proc Natl Acad Sci U S A (1999) 7.60
Correlation between severity and SMN protein level in spinal muscular atrophy. Nat Genet (1997) 6.08
The SMN-SIP1 complex has an essential role in spliceosomal snRNP biogenesis. Cell (1997) 5.57
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A transgene carrying an A2G missense mutation in the SMN gene modulates phenotypic severity in mice with severe (type I) spinal muscular atrophy. J Cell Biol (2003) 1.75
Therapeutics development for spinal muscular atrophy. NeuroRx (2006) 1.62
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A Drosophila melanogaster model of spinal muscular atrophy reveals a function for SMN in striated muscle. J Cell Biol (2007) 1.60
Autoimmune channelopathies and related neurological disorders. Neuron (2006) 1.56
Evidence for a modifying pathway in SMA discordant families: reduced SMN level decreases the amount of its interacting partners and Htra2-beta1. Hum Genet (2003) 1.52
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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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Rescue of the spinal muscular atrophy phenotype in a mouse model by early postnatal delivery of SMN. Nat Biotechnol (2010) 4.62
Astrocytes from familial and sporadic ALS patients are toxic to motor neurons. Nat Biotechnol (2011) 3.45
Trichostatin A increases SMN expression and survival in a mouse model of spinal muscular atrophy. J Clin Invest (2007) 3.11
Knockdown of the survival motor neuron (Smn) protein in zebrafish causes defects in motor axon outgrowth and pathfinding. J Cell Biol (2003) 2.94
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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
Valproic acid increases SMN levels in spinal muscular atrophy patient cells. Ann Neurol (2003) 2.28
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A single administration of morpholino antisense oligomer rescues spinal muscular atrophy in mouse. Hum Mol Genet (2011) 2.13
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Abnormal motor phenotype in the SMNDelta7 mouse model of spinal muscular atrophy. Neurobiol Dis (2007) 2.10
An SMN-dependent U12 splicing event essential for motor circuit function. Cell (2012) 1.94
Early heart failure in the SMNDelta7 model of spinal muscular atrophy and correction by postnatal scAAV9-SMN delivery. Hum Mol Genet (2010) 1.89
Embryonic motor axon development in the severe SMA mouse. Hum Mol Genet (2008) 1.79
A transgene carrying an A2G missense mutation in the SMN gene modulates phenotypic severity in mice with severe (type I) spinal muscular atrophy. J Cell Biol (2003) 1.75
Lentivector-mediated SMN replacement in a mouse model of spinal muscular atrophy. J Clin Invest (2004) 1.73
A positive modifier of spinal muscular atrophy in the SMN2 gene. Am J Hum Genet (2009) 1.70
SMN is required for sensory-motor circuit function in Drosophila. Cell (2012) 1.68
Postsymptomatic restoration of SMN rescues the disease phenotype in a mouse model of severe spinal muscular atrophy. J Clin Invest (2011) 1.62
The activity of the spinal muscular atrophy protein is regulated during development and cellular differentiation. Hum Mol Genet (2005) 1.61
NF-κB-mediated Pax7 dysregulation in the muscle microenvironment promotes cancer cachexia. J Clin Invest (2013) 1.60
Identification and characterization of Gemin7, a novel component of the survival of motor neuron complex. J Biol Chem (2002) 1.60
Increased expression of the glial glutamate transporter EAAT2 modulates excitotoxicity and delays the onset but not the outcome of ALS in mice. Hum Mol Genet (2003) 1.53
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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
Temporal requirement for high SMN expression in SMA mice. Hum Mol Genet (2011) 1.44
The SMN complex is associated with snRNPs throughout their cytoplasmic assembly pathway. Mol Cell Biol (2002) 1.39
A genetic model of amyotrophic lateral sclerosis in zebrafish displays phenotypic hallmarks of motoneuron disease. Dis Model Mech (2010) 1.36
snRNAs contain specific SMN-binding domains that are essential for snRNP assembly. Mol Cell Biol (2004) 1.34
Gemin8 is a novel component of the survival motor neuron complex and functions in small nuclear ribonucleoprotein assembly. J Biol Chem (2006) 1.34
Protein phosphatase 1 binds to the RNA recognition motif of several splicing factors and regulates alternative pre-mRNA processing. Hum Mol Genet (2007) 1.33
Protein- and mRNA-based phenotype-genotype correlations in DMD/BMD with point mutations and molecular basis for BMD with nonsense and frameshift mutations in the DMD gene. Hum Mutat (2007) 1.31
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
Unrip is a component of SMN complexes active in snRNP assembly. FEBS Lett (2005) 1.28
La protein is associated with terminal oligopyrimidine mRNAs in actively translating polysomes. J Biol Chem (2003) 1.19
A role for SMN exon 7 splicing in the selective vulnerability of motor neurons in spinal muscular atrophy. Mol Cell Biol (2011) 1.16
Translational control of glial glutamate transporter EAAT2 expression. J Biol Chem (2006) 1.14
Gemin8 is required for the architecture and function of the survival motor neuron complex. J Biol Chem (2006) 1.10
Synthesis and biological evaluation of novel 2,4-diaminoquinazoline derivatives as SMN2 promoter activators for the potential treatment of spinal muscular atrophy. J Med Chem (2008) 1.10
SMN complex localizes to the sarcomeric Z-disc and is a proteolytic target of calpain. Hum Mol Genet (2008) 1.07
Identification of a novel cyclic AMP-response element (CRE-II) and the role of CREB-1 in the cAMP-induced expression of the survival motor neuron (SMN) gene. J Biol Chem (2004) 1.03
Spinal muscular atrophy: development and implementation of potential treatments. Ann Neurol (2013) 1.01
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
Improved antisense oligonucleotide design to suppress aberrant SMN2 gene transcript processing: towards a treatment for spinal muscular atrophy. PLoS One (2013) 1.00
The DcpS inhibitor RG3039 improves motor function in SMA mice. Hum Mol Genet (2013) 0.99
Absence of gemin5 from SMN complexes in nuclear Cajal bodies. BMC Cell Biol (2007) 0.98
Antisense oligonucleotides for the treatment of spinal muscular atrophy. Hum Gene Ther (2013) 0.95
Human glioma cells and undifferentiated primary astrocytes that express aberrant EAAT2 mRNA inhibit normal EAAT2 protein expression and prevent cell death. Mol Cell Neurosci (2002) 0.94
The SMN binding protein Gemin2 is not involved in motor axon outgrowth. Dev Neurobiol (2008) 0.91
A large animal model of spinal muscular atrophy and correction of phenotype. Ann Neurol (2015) 0.90
IPLEX administration improves motor neuron survival and ameliorates motor functions in a severe mouse model of spinal muscular atrophy. Mol Med (2012) 0.88
MiR-21 is an Ngf-modulated microRNA that supports Ngf signaling and regulates neuronal degeneration in PC12 cells. Neuromolecular Med (2014) 0.87
Detection of human survival motor neuron (SMN) protein in mice containing the SMN2 transgene: applicability to preclinical therapy development for spinal muscular atrophy. J Neurosci Methods (2008) 0.87
A cell system for phenotypic screening of modifiers of SMN2 gene expression and function. PLoS One (2013) 0.80
SMN deficiency disrupts gastrointestinal and enteric nervous system function in mice. Hum Mol Genet (2015) 0.77
Advances in modeling and treating spinal muscular atrophy. Curr Opin Neurol (2016) 0.75
Let all DNA vote: who are the amyotrophic lateral sclerosis candidates? Neurology (2008) 0.75