Published in Results Probl Cell Differ on January 01, 2009
Temporal requirement for high SMN expression in SMA mice. Hum Mol Genet (2011) 1.44
Spinal muscular atrophy: the role of SMN in axonal mRNA regulation. Brain Res (2012) 1.26
The survival of motor neuron (SMN) protein interacts with the mRNA-binding protein HuD and regulates localization of poly(A) mRNA in primary motor neuron axons. J Neurosci (2011) 1.25
The contribution of mouse models to understanding the pathogenesis of spinal muscular atrophy. Dis Model Mech (2011) 1.15
Survival motor neuron affects plastin 3 protein levels leading to motor defects. J Neurosci (2012) 1.07
High-efficiency transfection of cultured primary motor neurons to study protein localization, trafficking, and function. Mol Neurodegener (2010) 1.02
Spinal muscular atrophy: development and implementation of potential treatments. Ann Neurol (2013) 1.01
Splicing therapy for neuromuscular disease. Mol Cell Neurosci (2013) 0.95
mRNA localization: an orchestration of assembly, traffic and synthesis. Traffic (2012) 0.94
Dynamics of survival of motor neuron (SMN) protein interaction with the mRNA-binding protein IMP1 facilitates its trafficking into motor neuron axons. Dev Neurobiol (2013) 0.91
SMN-inducing compounds for the treatment of spinal muscular atrophy. Future Med Chem (2012) 0.90
Ubiquitin-specific protease 9x deubiquitinates and stabilizes the spinal muscular atrophy protein-survival motor neuron. J Biol Chem (2012) 0.88
Molecular determinants of survival motor neuron (SMN) protein cleavage by the calcium-activated protease, calpain. PLoS One (2010) 0.84
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
Presynaptic localization of Smn and hnRNP R in axon terminals of embryonic and postnatal mouse motoneurons. PLoS One (2014) 0.81
Implication of the SMN complex in the biogenesis and steady state level of the signal recognition particle. Nucleic Acids Res (2012) 0.80
Biomarkers in rare disorders: the experience with spinal muscular atrophy. Int J Mol Sci (2010) 0.79
Splicing inhibition of U2AF65 leads to alternative exon skipping. Proc Natl Acad Sci U S A (2015) 0.79
Large-Scale Production of Adeno-Associated Viral Vector Serotype-9 Carrying the Human Survival Motor Neuron Gene. Mol Biotechnol (2016) 0.78
Structural basis for specific recognition of pre-snRNA by Gemin5. Cell Res (2016) 0.78
Conserved requirement for DEAD-box RNA helicase Gemin3 in Drosophila oogenesis. BMC Res Notes (2012) 0.78
Identification of the phosphorylation sites in the survival motor neuron protein by protein kinase A. Biochim Biophys Acta (2011) 0.77
GEMINs: potential therapeutic targets for spinal muscular atrophy? Front Neurosci (2014) 0.77
Insights into the evolutionary features of human neurodegenerative diseases. PLoS One (2012) 0.76
In vitro gene manipulation of spinal muscular atrophy fibroblast cell line using gene-targeting fragment for restoration of SMN protein expression. Gene Ther (2015) 0.75
Dysregulation of mRNA Localization and Translation in Genetic Disease. J Neurosci (2016) 0.75
Smn-Deficiency Increases the Intrinsic Excitability of Motoneurons. Front Cell Neurosci (2017) 0.75
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Stress granules and processing bodies are dynamically linked sites of mRNP remodeling. J Cell Biol (2005) 9.25
RNA granules. J Cell Biol (2006) 7.79
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
Intravascular AAV9 preferentially targets neonatal neurons and adult astrocytes. Nat Biotechnol (2008) 6.82
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Stress granules: the Tao of RNA triage. Trends Biochem Sci (2008) 6.27
The SMN-SIP1 complex has an essential role in spliceosomal snRNP biogenesis. Cell (1997) 5.57
The human centromeric survival motor neuron gene (SMN2) rescues embryonic lethality in Smn(-/-) mice and results in a mouse with spinal muscular atrophy. Hum Mol Genet (2000) 5.38
Spatial regulation of beta-actin translation by Src-dependent phosphorylation of ZBP1. Nature (2005) 5.29
The spinal muscular atrophy disease gene product, SMN, and its associated protein SIP1 are in a complex with spliceosomal snRNP proteins. Cell (1997) 5.28
SMNDelta7, the major product of the centromeric survival motor neuron (SMN2) gene, extends survival in mice with spinal muscular atrophy and associates with full-length SMN. Hum Mol Genet (2005) 5.23
Characterization of a beta-actin mRNA zipcode-binding protein. Mol Cell Biol (1997) 5.21
A novel nuclear structure containing the survival of motor neurons protein. EMBO J (1996) 4.72
Inactivation of the survival motor neuron gene, a candidate gene for human spinal muscular atrophy, leads to massive cell death in early mouse embryos. Proc Natl Acad Sci U S A (1997) 4.70
SMN deficiency causes tissue-specific perturbations in the repertoire of snRNAs and widespread defects in splicing. Cell (2008) 4.54
Essential role for the SMN complex in the specificity of snRNP assembly. Science (2002) 4.42
A mouse model for spinal muscular atrophy. Nat Genet (2000) 4.37
Mass spectrometry and EST-database searching allows characterization of the multi-protein spliceosome complex. Nat Genet (1998) 4.16
SMN tudor domain structure and its interaction with the Sm proteins. Nat Struct Biol (2001) 4.03
A new regulatory protein, KSRP, mediates exon inclusion through an intronic splicing enhancer. Genes Dev (1997) 3.88
Asymmetrical beta-actin mRNA translation in growth cones mediates attractive turning to netrin-1. Nat Neurosci (2006) 3.74
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Identifying specific protein interaction partners using quantitative mass spectrometry and bead proteomes. J Cell Biol (2008) 3.53
SMN oligomerization defect correlates with spinal muscular atrophy severity. Nat Genet (1998) 3.51
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Neuronal RNA granules: movers and makers. Neuron (2006) 3.44
An exonic enhancer is required for inclusion of an essential exon in the SMA-determining gene SMN. Hum Mol Genet (2000) 3.41
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
A multiprotein complex mediates the ATP-dependent assembly of spliceosomal U snRNPs. Nat Cell Biol (2001) 3.39
An essential role for beta-actin mRNA localization and translation in Ca2+-dependent growth cone guidance. Nat Neurosci (2006) 3.30
Dendritic mRNA: transport, translation and function. Nat Rev Neurosci (2007) 3.29
The methylosome, a 20S complex containing JBP1 and pICln, produces dimethylarginine-modified Sm proteins. Mol Cell Biol (2001) 3.27
Antisense masking of an hnRNP A1/A2 intronic splicing silencer corrects SMN2 splicing in transgenic mice. Am J Hum Genet (2008) 3.24
Selective vulnerability of motor neurons and dissociation of pre- and post-synaptic pathology at the neuromuscular junction in mouse models of spinal muscular atrophy. Hum Mol Genet (2007) 3.09
SMN, the product of the spinal muscular atrophy gene, binds preferentially to dimethylarginine-containing protein targets. Mol Cell (2001) 2.95
Knockdown of the survival motor neuron (Smn) protein in zebrafish causes defects in motor axon outgrowth and pathfinding. J Cell Biol (2003) 2.94
Gemin3: A novel DEAD box protein that interacts with SMN, the spinal muscular atrophy gene product, and is a component of gems. J Cell Biol (1999) 2.93
Spinal muscular atrophy. Lancet (2008) 2.92
The SMN complex, an assemblyosome of ribonucleoproteins. Curr Opin Cell Biol (2002) 2.88
De novo and inherited deletions of the 5q13 region in spinal muscular atrophies. Science (1994) 2.87
A mechanism for translationally coupled mRNA turnover: interaction between the poly(A) tail and a c-fos RNA coding determinant via a protein complex. Cell (2000) 2.83
Reduced SMN protein impairs maturation of the neuromuscular junctions in mouse models of spinal muscular atrophy. Hum Mol Genet (2008) 2.82
Gemin4. A novel component of the SMN complex that is found in both gems and nucleoli. J Cell Biol (2000) 2.78
An update of the mutation spectrum of the survival motor neuron gene (SMN1) in autosomal recessive spinal muscular atrophy (SMA). Hum Mutat (2000) 2.71
Impaired synaptic vesicle release and immaturity of neuromuscular junctions in spinal muscular atrophy mice. J Neurosci (2009) 2.71
Ribonucleoprotein assembly defects correlate with spinal muscular atrophy severity and preferentially affect a subset of spliceosomal snRNPs. PLoS One (2007) 2.70
Neurotrophin-induced transport of a beta-actin mRNP complex increases beta-actin levels and stimulates growth cone motility. Neuron (2001) 2.57
SMN interacts with a novel family of hnRNP and spliceosomal proteins. EMBO J (2001) 2.51
Plastin 3 is a protective modifier of autosomal recessive spinal muscular atrophy. Science (2008) 2.51
Translational control of localized mRNAs: restricting protein synthesis in space and time. Nat Rev Mol Cell Biol (2008) 2.50
A functional interaction between the survival motor neuron complex and RNA polymerase II. J Cell Biol (2001) 2.44
SMN-mediated assembly of RNPs: a complex story. Trends Cell Biol (2002) 2.37
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
The survival of motor neurons (SMN) protein interacts with the snoRNP proteins fibrillarin and GAR1. Curr Biol (2001) 2.29
Active transport of the survival motor neuron protein and the role of exon-7 in cytoplasmic localization. J Neurosci (2003) 2.28
Gemin5, a novel WD repeat protein component of the SMN complex that binds Sm proteins. J Biol Chem (2001) 2.25
Tudor domains bind symmetrical dimethylated arginines. J Biol Chem (2005) 2.23
Reduced U snRNP assembly causes motor axon degeneration in an animal model for spinal muscular atrophy. Genes Dev (2005) 2.22
SMN mutants of spinal muscular atrophy patients are defective in binding to snRNP proteins. Proc Natl Acad Sci U S A (1999) 2.18
Translation elongation factor 1A is essential for regulation of the actin cytoskeleton and cell morphology. Nat Struct Mol Biol (2005) 2.15
Neurofilament accumulation at the motor endplate and lack of axonal sprouting in a spinal muscular atrophy mouse model. Hum Mol Genet (2002) 2.13
Survival motor neuron function in motor axons is independent of functions required for small nuclear ribonucleoprotein biogenesis. J Neurosci (2006) 2.11
Nuclear targeting defect of SMN lacking the C-terminus in a mouse model of spinal muscular atrophy. Hum Mol Genet (2000) 2.09
Why do cells need an assembly machine for RNA-protein complexes? Trends Cell Biol (2004) 2.05
Specific interaction of Smn, the spinal muscular atrophy determining gene product, with hnRNP-R and gry-rbp/hnRNP-Q: a role for Smn in RNA processing in motor axons? Hum Mol Genet (2002) 2.05
Assisted RNP assembly: SMN and PRMT5 complexes cooperate in the formation of spliceosomal UsnRNPs. EMBO J (2002) 2.04
Real-time visualization of ZBP1 association with beta-actin mRNA during transcription and localization. Curr Biol (2003) 2.04
Coilin forms the bridge between Cajal bodies and SMN, the spinal muscular atrophy protein. Genes Dev (2001) 2.03
Regulation of SMN protein stability. Mol Cell Biol (2008) 2.02
Direct interaction of the spinal muscular atrophy disease protein SMN with the small nucleolar RNA-associated protein fibrillarin. J Biol Chem (2001) 2.00
Spinal muscular atrophy disrupts the interaction of ZPR1 with the SMN protein. Nat Cell Biol (2001) 2.00
Neurotrophin regulation of beta-actin mRNA and protein localization within growth cones. J Cell Biol (1999) 1.99
Higher order arrangement of the eukaryotic nuclear bodies. Proc Natl Acad Sci U S A (2002) 1.99
The SMN complex. Exp Cell Res (2004) 1.96
RNA transport and local control of translation. Annu Rev Cell Dev Biol (2005) 1.96
Inhibiting axon degeneration and synapse loss attenuates apoptosis and disease progression in a mouse model of motoneuron disease. Curr Biol (2003) 1.92
Coilin methylation regulates nuclear body formation. Dev Cell (2002) 1.91
Dendritic localization of the translational repressor Pumilio 2 and its contribution to dendritic stress granules. J Neurosci (2006) 1.90
The relationship between SMN, the spinal muscular atrophy protein, and nuclear coiled bodies in differentiated tissues and cultured cells. Exp Cell Res (2000) 1.89
RNA-associated protein 55 (RAP55) localizes to mRNA processing bodies and stress granules. RNA (2006) 1.89
The changing natural history of spinal muscular atrophy type 1. Neurology (2007) 1.87
Survival motor neuron protein facilitates assembly of stress granules. FEBS Lett (2004) 1.86
Neuromuscular defects in a Drosophila survival motor neuron gene mutant. Hum Mol Genet (2003) 1.85
ZBP1 regulates mRNA stability during cellular stress. J Cell Biol (2006) 1.84
High-resolution X-ray and NMR structures of the SMN Tudor domain: conformational variation in the binding site for symmetrically dimethylated arginine residues. J Mol Biol (2003) 1.84
Symmetrical dimethylarginine methylation is required for the localization of SMN in Cajal bodies and pre-mRNA splicing. J Cell Biol (2002) 1.83
SMN gene duplication and the emergence of the SMN2 gene occurred in distinct hominids: SMN2 is unique to Homo sapiens. Hum Genet (2001) 1.81
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
The SMN complex: an assembly machine for RNPs. Cold Spring Harb Symp Quant Biol (2006) 1.79
Spatial regulation of beta-actin translation by Src-dependent phosphorylation of ZBP1. Nature (2005) 5.29
SMNDelta7, the major product of the centromeric survival motor neuron (SMN2) gene, extends survival in mice with spinal muscular atrophy and associates with full-length SMN. Hum Mol Genet (2005) 5.23
Neuronal RNA granules: movers and makers. Neuron (2006) 3.44
Dysregulated metabotropic glutamate receptor-dependent translation of AMPA receptor and postsynaptic density-95 mRNAs at synapses in a mouse model of fragile X syndrome. J Neurosci (2007) 3.31
An essential role for beta-actin mRNA localization and translation in Ca2+-dependent growth cone guidance. Nat Neurosci (2006) 3.30
Metabotropic glutamate receptor activation regulates fragile x mental retardation protein and FMR1 mRNA localization differentially in dendrites and at synapses. J Neurosci (2004) 2.59
Extracellular stimuli specifically regulate localized levels of individual neuronal mRNAs. J Cell Biol (2007) 2.54
A direct role for FMRP in activity-dependent dendritic mRNA transport links filopodial-spine morphogenesis to fragile X syndrome. Dev Cell (2008) 2.51
Plastin 3 is a protective modifier of autosomal recessive spinal muscular atrophy. Science (2008) 2.51
Activity-dependent trafficking and dynamic localization of zipcode binding protein 1 and beta-actin mRNA in dendrites and spines of hippocampal neurons. J Neurosci (2003) 2.34
Active transport of the survival motor neuron protein and the role of exon-7 in cytoplasmic localization. J Neurosci (2003) 2.28
Reversible inhibition of PSD-95 mRNA translation by miR-125a, FMRP phosphorylation, and mGluR signaling. Mol Cell (2011) 2.27
Survival motor neuron function in motor axons is independent of functions required for small nuclear ribonucleoprotein biogenesis. J Neurosci (2006) 2.11
Fragile X mental retardation protein deficiency leads to excessive mGluR5-dependent internalization of AMPA receptors. Proc Natl Acad Sci U S A (2007) 2.10
Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis. Nature (2012) 2.08
FMRP phosphorylation reveals an immediate-early signaling pathway triggered by group I mGluR and mediated by PP2A. J Neurosci (2007) 2.02
Excess phosphoinositide 3-kinase subunit synthesis and activity as a novel therapeutic target in fragile X syndrome. J Neurosci (2010) 1.89
Local functions for FMRP in axon growth cone motility and activity-dependent regulation of filopodia and spine synapses. Mol Cell Neurosci (2006) 1.88
S6K1 phosphorylates and regulates fragile X mental retardation protein (FMRP) with the neuronal protein synthesis-dependent mammalian target of rapamycin (mTOR) signaling cascade. J Biol Chem (2008) 1.87
Local RNA translation at the synapse and in disease. J Neurosci (2011) 1.83
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
A predominantly nuclear protein affecting cytoplasmic localization of beta-actin mRNA in fibroblasts and neurons. J Cell Biol (2002) 1.77
Localization of a beta-actin messenger ribonucleoprotein complex with zipcode-binding protein modulates the density of dendritic filopodia and filopodial synapses. J Neurosci (2003) 1.74
Activity bidirectionally regulates AMPA receptor mRNA abundance in dendrites of hippocampal neurons. J Neurosci (2006) 1.60
Limited availability of ZBP1 restricts axonal mRNA localization and nerve regeneration capacity. EMBO J (2011) 1.49
Single molecule-sensitive probes for imaging RNA in live cells. Nat Methods (2009) 1.40
Netrin-1-induced local β-actin synthesis and growth cone guidance requires zipcode binding protein 1. J Neurosci (2011) 1.32
Therapeutic strategies in fragile X syndrome: dysregulated mGluR signaling and beyond. Neuropsychopharmacology (2011) 1.30
Fragile X mental retardation protein regulates protein expression and mRNA translation of the potassium channel Kv4.2. J Neurosci (2011) 1.30
Phosphorylation of zipcode binding protein 1 is required for brain-derived neurotrophic factor signaling of local beta-actin synthesis and growth cone turning. J Neurosci (2010) 1.29
Bidirectional control of mRNA translation and synaptic plasticity by the cytoplasmic polyadenylation complex. Mol Cell (2012) 1.29
Axonal Localization of transgene mRNA in mature PNS and CNS neurons. J Neurosci (2011) 1.28
Spinal muscular atrophy: the role of SMN in axonal mRNA regulation. Brain Res (2012) 1.26
The ALS disease protein TDP-43 is actively transported in motor neuron axons and regulates axon outgrowth. Hum Mol Genet (2012) 1.25
The survival of motor neuron (SMN) protein interacts with the mRNA-binding protein HuD and regulates localization of poly(A) mRNA in primary motor neuron axons. J Neurosci (2011) 1.25
Dephosphorylation-induced ubiquitination and degradation of FMRP in dendrites: a role in immediate early mGluR-stimulated translation. J Neurosci (2012) 1.23
GAP-43 mRNA in growth cones is associated with HuD and ribosomes. J Neurobiol (2004) 1.21
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Making and breaking synapses through local mRNA regulation. Curr Opin Genet Dev (2011) 1.17
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Mutation of the conserved polyadenosine RNA binding protein, ZC3H14/dNab2, impairs neural function in Drosophila and humans. Proc Natl Acad Sci U S A (2011) 1.11
Dynamic association of the fragile X mental retardation protein as a messenger ribonucleoprotein between microtubules and polyribosomes. Mol Biol Cell (2007) 1.10
Fragile X Mental Retardation Protein is Involved in Protein Synthesis-Dependent Collapse of Growth Cones Induced by Semaphorin-3A. Front Neural Circuits (2009) 1.08
The COPI vesicle complex binds and moves with survival motor neuron within axons. Hum Mol Genet (2011) 1.08
Coaggregation of RNA-binding proteins in a model of TDP-43 proteinopathy with selective RGG motif methylation and a role for RRM1 ubiquitination. PLoS One (2012) 1.05
Genetic and acute CPEB1 depletion ameliorate fragile X pathophysiology. Nat Med (2013) 1.04
High-efficiency transfection of cultured primary motor neurons to study protein localization, trafficking, and function. Mol Neurodegener (2010) 1.02
CPEB4 is a cell survival protein retained in the nucleus upon ischemia or endoplasmic reticulum calcium depletion. Mol Cell Biol (2010) 1.01
Experience-induced Arc/Arg3.1 primes CA1 pyramidal neurons for metabotropic glutamate receptor-dependent long-term synaptic depression. Neuron (2013) 1.00
Automated 4D analysis of dendritic spine morphology: applications to stimulus-induced spine remodeling and pharmacological rescue in a disease model. Mol Brain (2011) 0.98
Excess protein synthesis in FXS patient lymphoblastoid cells can be rescued with a p110β-selective inhibitor. Mol Med (2012) 0.96
Negative regulation of RhoA translation and signaling by hnRNP-Q1 affects cellular morphogenesis. Mol Biol Cell (2012) 0.94
mRNA localization: an orchestration of assembly, traffic and synthesis. Traffic (2012) 0.94
A boost in microRNAs shapes up the neuron. EMBO J (2009) 0.93
Identification of axon-enriched microRNAs localized to growth cones of cortical neurons. Dev Neurobiol (2013) 0.93
RACK1 is a ribosome scaffold protein for β-actin mRNA/ZBP1 complex. PLoS One (2012) 0.91
Dendritic GluN2A synthesis mediates activity-induced NMDA receptor insertion. J Neurosci (2013) 0.90
QNQKE targeting motif for the SMN-Gemin multiprotein complexin neurons. J Neurosci Res (2007) 0.90
Therapeutic Strategies in Fragile X Syndrome: From Bench to Bedside and Back. Neurotherapeutics (2015) 0.90
FMRP interacts with G-quadruplex structures in the 3'-UTR of its dendritic target Shank1 mRNA. RNA Biol (2014) 0.89
MeCP2 regulates the synaptic expression of a Dysbindin-BLOC-1 network component in mouse brain and human induced pluripotent stem cell-derived neurons. PLoS One (2013) 0.87
High-resolution fluorescence in situ hybridization to detect mRNAs in neuronal compartments in vitro and in vivo. Methods Mol Biol (2011) 0.86
Regulation of zipcode binding protein 1 transport dynamics in axons by myosin Va. J Neurosci (2012) 0.86
Regulation of chemotropic guidance of nerve growth cones by microRNA. Mol Brain (2011) 0.85
Reducing glutamate signaling pays off in fragile X. Nat Med (2008) 0.85
Poly(A) RNA-binding proteins and polyadenosine RNA: new members and novel functions. Wiley Interdiscip Rev RNA (2014) 0.83
Fragile balance: RNA editing tunes the synapse. Nat Neurosci (2011) 0.77