Published in Curr Opin Cell Biol on June 01, 2002
Numerous microRNPs in neuronal cells containing novel microRNAs. RNA (2003) 6.28
SMN deficiency causes tissue-specific perturbations in the repertoire of snRNAs and widespread defects in splicing. Cell (2008) 4.54
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
miRNP:mRNA association in polyribosomes in a human neuronal cell line. RNA (2004) 3.39
Ribonucleoprotein assembly defects correlate with spinal muscular atrophy severity and preferentially affect a subset of spliceosomal snRNPs. PLoS One (2007) 2.70
Human telomerase RNA and box H/ACA scaRNAs share a common Cajal body-specific localization signal. J Cell Biol (2004) 2.49
A role for Q/N-rich aggregation-prone regions in P-body localization. J Cell Sci (2008) 2.30
Telomerase RNA accumulates in Cajal bodies in human cancer cells. Mol Biol Cell (2003) 2.14
Unique Sm core structure of U7 snRNPs: assembly by a specialized SMN complex and the role of a new component, Lsm11, in histone RNA processing. Genes Dev (2003) 2.10
HIV-1 nef suppression by virally encoded microRNA. Retrovirology (2004) 2.05
Regulation of SMN protein stability. Mol Cell Biol (2008) 2.02
In vivo kinetics of Cajal body components. J Cell Biol (2004) 1.91
The Cajal body: a meeting place for spliceosomal snRNPs in the nuclear maze. Chromosoma (2006) 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
Chaperoning ribonucleoprotein biogenesis in health and disease. EMBO Rep (2007) 1.77
Modeling spinal muscular atrophy in Drosophila. PLoS One (2008) 1.71
Cajal bodies and coilin--moving towards function. J Cell Biol (2002) 1.69
Therapeutics development for spinal muscular atrophy. NeuroRx (2006) 1.62
Targeting of U4/U6 small nuclear RNP assembly factor SART3/p110 to Cajal bodies. J Cell Biol (2003) 1.61
Two distinct arginine methyltransferases are required for biogenesis of Sm-class ribonucleoproteins. J Cell Biol (2007) 1.58
SMN, the spinal muscular atrophy protein, forms a pre-import snRNP complex with snurportin1 and importin beta. Hum Mol Genet (2002) 1.36
Interaction between the small-nuclear-RNA cap hypermethylase and the spinal muscular atrophy protein, survival of motor neuron. EMBO Rep (2003) 1.35
Novel aminoglycosides increase SMN levels in spinal muscular atrophy fibroblasts. Hum Genet (2006) 1.31
Intact satellite cells lead to remarkable protection against Smn gene defect in differentiated skeletal muscle. J Cell Biol (2003) 1.29
Structural basis for dimethylarginine recognition by the Tudor domains of human SMN and SPF30 proteins. Nat Struct Mol Biol (2011) 1.29
Gemin5 delivers snRNA precursors to the SMN complex for snRNP biogenesis. Mol Cell (2010) 1.27
Spinal muscular atrophy: the role of SMN in axonal mRNA regulation. Brain Res (2012) 1.26
Cajal bodies and the nucleolus are required for a plant virus systemic infection. EMBO J (2007) 1.25
Deletion of murine Smn exon 7 directed to liver leads to severe defect of liver development associated with iron overload. Am J Pathol (2004) 1.24
U bodies are cytoplasmic structures that contain uridine-rich small nuclear ribonucleoproteins and associate with P bodies. Proc Natl Acad Sci U S A (2007) 1.21
Depletion of SMN by RNA interference in HeLa cells induces defects in Cajal body formation. Nucleic Acids Res (2006) 1.19
Developments in RNA splicing and disease. Cold Spring Harb Perspect Biol (2011) 1.18
Spinal muscular atrophy and a model for survival of motor neuron protein function in axonal ribonucleoprotein complexes. Results Probl Cell Differ (2009) 1.17
A feedback loop regulates splicing of the spinal muscular atrophy-modifying gene, SMN2. Hum Mol Genet (2010) 1.15
The C-terminal domain of coilin interacts with Sm proteins and U snRNPs. Chromosoma (2005) 1.13
Spliceosomal small nuclear ribonucleoprotein particles repeatedly cycle through Cajal bodies. Mol Biol Cell (2008) 1.11
Mutations in PRPF31 inhibit pre-mRNA splicing of rhodopsin gene and cause apoptosis of retinal cells. J Neurosci (2005) 1.09
Survival motor neuron affects plastin 3 protein levels leading to motor defects. J Neurosci (2012) 1.07
Archaeal and eukaryotic homologs of Hfq: A structural and evolutionary perspective on Sm function. RNA Biol (2013) 1.07
Cotranslational assembly of the yeast SET1C histone methyltransferase complex. EMBO J (2009) 1.06
Proteomic assessment of a cell model of spinal muscular atrophy. BMC Neurosci (2011) 1.05
Biochemical and genetic evidence for a role of IGHMBP2 in the translational machinery. Hum Mol Genet (2009) 1.02
Assembly of the U1 snRNP involves interactions with the backbone of the terminal stem of U1 snRNA. RNA (2003) 1.01
Genetically engineered pig models for human diseases. Annu Rev Anim Biosci (2013) 1.01
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
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
SMN-assisted assembly of snRNP-specific Sm cores in trypanosomes. Genes Dev (2009) 0.99
Restoration of full-length SMN promoted by adenoviral vectors expressing RNA antisense oligonucleotides embedded in U7 snRNAs. PLoS One (2009) 0.98
A novel function for human factor C1 (HCF-1), a host protein required for herpes simplex virus infection, in pre-mRNA splicing. EMBO J (2002) 0.98
Absence of gemin5 from SMN complexes in nuclear Cajal bodies. BMC Cell Biol (2007) 0.98
The Role of Protein Arginine Methylation in mRNP Dynamics. Mol Biol Int (2011) 0.98
Sm protein methylation is dispensable for snRNP assembly in Drosophila melanogaster. RNA (2008) 0.97
A SMNDelta7 read-through product confers functionality to the SMNDelta7 protein. Neurosci Lett (2008) 0.97
Modeling spinal muscular atrophy in Drosophila links Smn to FGF signaling. J Cell Biol (2011) 0.96
More than a bystander: the contributions of intrinsic skeletal muscle defects in motor neuron diseases. Front Physiol (2013) 0.94
The exon junction complex component Y14 modulates the activity of the methylosome in biogenesis of spliceosomal small nuclear ribonucleoproteins. J Biol Chem (2011) 0.94
Understanding the biochemical activities of galectin-1 and galectin-3 in the nucleus. Glycoconj J (2004) 0.94
The FCP1 phosphatase interacts with RNA polymerase II and with MEP50 a component of the methylosome complex involved in the assembly of snRNP. Nucleic Acids Res (2003) 0.93
Specific splicing defects in S. pombe carrying a degron allele of the Survival of Motor Neuron gene. EMBO J (2010) 0.93
Evolutionary conservation and expression of human RNA-binding proteins and their role in human genetic disease. Adv Exp Med Biol (2014) 0.92
Spliceosomal small nuclear ribonucleoprotein biogenesis defects and motor neuron selectivity in spinal muscular atrophy. Brain Res (2012) 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
Identification of truncated forms of U1 snRNA reveals a novel RNA degradation pathway during snRNP biogenesis. Nucleic Acids Res (2013) 0.90
SMN deficiency reduces cellular ability to form stress granules, sensitizing cells to stress. Cell Mol Neurobiol (2011) 0.90
Splicing therapeutics in SMN2 and APOB. Curr Opin Mol Ther (2009) 0.89
Repair of pre-mRNA splicing: prospects for a therapy for spinal muscular atrophy. RNA Biol (2010) 0.89
A novel germ cell-specific protein, SHIP1, forms a complex with chromatin remodeling activity during spermatogenesis. J Biol Chem (2008) 0.89
Ubiquitin-specific protease 9x deubiquitinates and stabilizes the spinal muscular atrophy protein-survival motor neuron. J Biol Chem (2012) 0.88
Therapeutic developments in spinal muscular atrophy. Ther Adv Neurol Disord (2010) 0.87
Aging--RNA in development and disease. Wiley Interdiscip Rev RNA (2011) 0.87
Solution structure of the core SMN-Gemin2 complex. Biochem J (2012) 0.86
Role of survival motor neuron complex components in small nuclear ribonucleoprotein assembly. J Biol Chem (2009) 0.86
Role of Cajal bodies and nucleolus in the maturation of the U1 snRNP in Arabidopsis. PLoS One (2008) 0.86
The survival motor neuron protein forms soluble glycine zipper oligomers. Structure (2012) 0.85
PSF contacts exon 7 of SMN2 pre-mRNA to promote exon 7 inclusion. Biochim Biophys Acta (2014) 0.85
Distal Alternative Last Exons Localize mRNAs to Neural Projections. Mol Cell (2016) 0.85
The Ighmbp2 helicase structure reveals the molecular basis for disease-causing mutations in DMSA1. Nucleic Acids Res (2012) 0.84
Molecular determinants of survival motor neuron (SMN) protein cleavage by the calcium-activated protease, calpain. PLoS One (2010) 0.84
Improvement of SMN2 pre-mRNA processing mediated by exon-specific U1 small nuclear RNA. Am J Hum Genet (2014) 0.84
Increased susceptibility of spinal muscular atrophy fibroblasts to camptothecin is p53-independent. BMC Cell Biol (2009) 0.83
Hypermethylated-capped selenoprotein mRNAs in mammals. Nucleic Acids Res (2014) 0.82
Fibroblast growth factor-2 regulates the stability of nuclear bodies. Proc Natl Acad Sci U S A (2009) 0.82
Reconstitution of the human U snRNP assembly machinery reveals stepwise Sm protein organization. EMBO J (2015) 0.82
Genome-wide identification of mRNAs associated with the protein SMN whose depletion decreases their axonal localization. RNA (2013) 0.82
Identifying and searching for conserved RNA localisation signals. Methods Mol Biol (2011) 0.81
Impaired Muscle Mitochondrial Biogenesis and Myogenesis in Spinal Muscular Atrophy. JAMA Neurol (2015) 0.81
Low levels of Survival Motor Neuron protein are sufficient for normal muscle function in the SMNΔ7 mouse model of SMA. Hum Mol Genet (2015) 0.81
Expression of Muscle-Specific MiRNA 206 in the Progression of Disease in a Murine SMA Model. PLoS One (2015) 0.81
Neurogenic and myogenic contributions to hereditary motor neuron disease. Neurodegener Dis (2012) 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
U1A regulates 3' processing of the survival motor neuron mRNA. J Biol Chem (2013) 0.79
Analysis of human protein replacement stable cell lines established using snoMEN-PR vector. PLoS One (2013) 0.79
Telomerase trafficking and assembly in Xenopus oocytes. J Cell Sci (2010) 0.78
Autoantibodies to survival of motor neuron complex in patients with polymyositis: immunoprecipitation of D, E, F, and G proteins without other components of small nuclear ribonucleoproteins. Arthritis Rheum (2011) 0.78
Signaling Over Distances. Mol Cell Proteomics (2015) 0.78
Organization of Plasmodium falciparum spliceosomal core complex and role of arginine methylation in its assembly. Malar J (2013) 0.78
Gemin5 Binds to the Survival Motor Neuron mRNA to Regulate SMN Expression. J Biol Chem (2015) 0.77
A novel functional role for MMSET in RNA processing based on the link between the REIIBP isoform and its interaction with the SMN complex. PLoS One (2014) 0.77
Cajal body proteins differentially affect the processing of box C/D scaRNPs. PLoS One (2015) 0.77
A uniform system for microRNA annotation. RNA (2003) 20.28
miRNPs: a novel class of ribonucleoproteins containing numerous microRNAs. Genes Dev (2002) 19.24
RNA and disease. Cell (2009) 7.98
Numerous microRNPs in neuronal cells containing novel microRNAs. RNA (2003) 6.28
Binding of a novel SMG-1-Upf1-eRF1-eRF3 complex (SURF) to the exon junction complex triggers Upf1 phosphorylation and nonsense-mediated mRNA decay. Genes Dev (2006) 5.59
RNA-binding proteins and post-transcriptional gene regulation. FEBS Lett (2008) 5.00
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
U1 snRNP protects pre-mRNAs from premature cleavage and polyadenylation. Nature (2010) 3.65
U1 snRNP determines mRNA length and regulates isoform expression. Cell (2012) 2.92
Translation is required to remove Y14 from mRNAs in the cytoplasm. Curr Biol (2002) 2.28
Ars2 links the nuclear cap-binding complex to RNA interference and cell proliferation. Cell (2009) 2.28
Gemin5, a novel WD repeat protein component of the SMN complex that binds Sm proteins. J Biol Chem (2001) 2.25
The survival of motor neurons protein determines the capacity for snRNP assembly: biochemical deficiency in spinal muscular atrophy. Mol Cell Biol (2005) 2.23
A novel WD repeat protein component of the methylosome binds Sm proteins. J Biol Chem (2001) 2.22
tRNA binds to cytochrome c and inhibits caspase activation. Mol Cell (2010) 2.14
Why do cells need an assembly machine for RNA-protein complexes? Trends Cell Biol (2004) 2.05
The SMN complex. Exp Cell Res (2004) 1.96
eIF4A3 is a novel component of the exon junction complex. RNA (2004) 1.93
Purification of native survival of motor neurons complexes and identification of Gemin6 as a novel component. J Biol Chem (2001) 1.73
The Gemin6-Gemin7 heterodimer from the survival of motor neurons complex has an Sm protein-like structure. Structure (2005) 1.64
Identification and characterization of Gemin7, a novel component of the survival of motor neuron complex. J Biol Chem (2002) 1.60
The Gemin5 protein of the SMN complex identifies snRNAs. Mol Cell (2006) 1.49
Sequence-specific interaction of U1 snRNA with the SMN complex. EMBO J (2002) 1.48
A simple whole cell lysate system for in vitro splicing reveals a stepwise assembly of the exon-exon junction complex. J Biol Chem (2003) 1.47
Molecular functions of the SMN complex. J Child Neurol (2007) 1.46
PYM binds the cytoplasmic exon-junction complex and ribosomes to enhance translation of spliced mRNAs. Nat Struct Mol Biol (2007) 1.45
Structure of the Y14-Magoh core of the exon junction complex. Curr Biol (2003) 1.43
Gemins modulate the expression and activity of the SMN complex. Hum Mol Genet (2005) 1.42
The SMN complex is associated with snRNPs throughout their cytoplasmic assembly pathway. Mol Cell Biol (2002) 1.39
Dysregulation of synaptogenesis genes antecedes motor neuron pathology in spinal muscular atrophy. Proc Natl Acad Sci U S A (2013) 1.35
snRNAs contain specific SMN-binding domains that are essential for snRNP assembly. Mol Cell Biol (2004) 1.34
Gemin5-snRNA interaction reveals an RNA binding function for WD repeat domains. Nat Struct Mol Biol (2009) 1.31
Detection of arginine dimethylated peptides by parallel precursor ion scanning mass spectrometry in positive ion mode. Anal Chem (2003) 1.29
Structure of a key intermediate of the SMN complex reveals Gemin2's crucial function in snRNP assembly. Cell (2011) 1.29
A degron created by SMN2 exon 7 skipping is a principal contributor to spinal muscular atrophy severity. Genes Dev (2010) 1.27
Gemin5 delivers snRNA precursors to the SMN complex for snRNP biogenesis. Mol Cell (2010) 1.27
Rapid-response splicing reporter screens identify differential regulators of constitutive and alternative splicing. Mol Cell Biol (2010) 1.18
Absence of heterogeneous nuclear ribonucleoproteins and survival motor neuron protein in TDP-43 positive inclusions in frontotemporal lobar degeneration. Acta Neuropathol (2007) 1.14
In vitro and in cellulo evidences for association of the survival of motor neuron complex with the fragile X mental retardation protein. J Biol Chem (2007) 1.08
Specific sequence features, recognized by the SMN complex, identify snRNAs and determine their fate as snRNPs. Mol Cell Biol (2005) 1.07
Inactivation of the SMN complex by oxidative stress. Mol Cell (2008) 1.06
A quantitative high-throughput in vitro splicing assay identifies inhibitors of spliceosome catalysis. Mol Cell Biol (2012) 1.05
A previously unidentified activity of yeast and mouse RNA:pseudouridine synthases 1 (Pus1p) on tRNAs. RNA (2006) 1.05
Where in the cell is the minor spliceosome? Proc Natl Acad Sci U S A (2008) 1.03
SMN-independent subunits of the SMN complex. Identification of a small nuclear ribonucleoprotein assembly intermediate. J Biol Chem (2007) 1.00
Genomics-guided discovery of thailanstatins A, B, and C As pre-mRNA splicing inhibitors and antiproliferative agents from Burkholderia thailandensis MSMB43. J Nat Prod (2013) 1.00
A novel cell immunoassay to measure survival of motor neurons protein in blood cells. BMC Neurol (2006) 1.00
Ars2 promotes proper replication-dependent histone mRNA 3' end formation. Mol Cell (2012) 0.99
Pseudouridylation at position 32 of mitochondrial and cytoplasmic tRNAs requires two distinct enzymes in Saccharomyces cerevisiae. J Biol Chem (2004) 0.98
Preparation of efficient splicing extracts from whole cells, nuclei, and cytoplasmic fractions. Methods Mol Biol (2008) 0.97
Minor introns are embedded molecular switches regulated by highly unstable U6atac snRNA. Elife (2013) 0.94
Lymphotropic Herpesvirus saimiri uses the SMN complex to assemble Sm cores on its small RNAs. Mol Cell Biol (2005) 0.94
Ce-Y14 and MAG-1, components of the exon-exon junction complex, are required for embryogenesis and germline sexual switching in Caenorhabditis elegans. Mech Dev (2004) 0.86
Specific Y14 domains mediate its nucleo-cytoplasmic shuttling and association with spliced mRNA. Sci Rep (2011) 0.83
Implication of the SMN complex in the biogenesis and steady state level of the signal recognition particle. Nucleic Acids Res (2012) 0.80