The survival of motor neurons protein determines the capacity for snRNP assembly: biochemical deficiency in spinal muscular atrophy.
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WRAP53 is essential for Cajal body formation and for targeting the survival of motor neuron complex to Cajal bodiesSMA CARNIVAL TRIAL PART II: a prospective, single-armed trial of L-carnitine and valproic acid in ambulatory children with spinal muscular atrophySMA CARNI-VAL trial part I: double-blind, randomized, placebo-controlled trial of L-carnitine and valproic acid in spinal muscular atrophyModeling spinal muscular atrophy in DrosophilaGemin8 is required for the architecture and function of the survival motor neuron complexGle1 functions during mRNA export in an oligomeric complex that is altered in human diseaseStructure of a key intermediate of the SMN complex reveals Gemin2's crucial function in snRNP assemblyTwo distinct arginine methyltransferases are required for biogenesis of Sm-class ribonucleoproteinsGemin proteins are required for efficient assembly of Sm-class ribonucleoproteins.The survival motor neuron protein forms soluble glycine zipper oligomersThe contribution of mouse models to understanding the pathogenesis of spinal muscular atrophyA SMN missense mutation complements SMN2 restoring snRNPs and rescuing SMA miceCopy Number Variations in the Survival Motor Neuron Genes: Implications for Spinal Muscular Atrophy and Other Neurodegenerative DiseasesSpinal muscular atrophy: development and implementation of potential treatmentsGemin5 Binds to the Survival Motor Neuron mRNA to Regulate SMN ExpressionGemin5 delivers snRNA precursors to the SMN complex for snRNP biogenesisGemin5-snRNA interaction reveals an RNA binding function for WD repeat domainsSMN-independent subunits of the SMN complex. Identification of a small nuclear ribonucleoprotein assembly intermediateA degron created by SMN2 exon 7 skipping is a principal contributor to spinal muscular atrophy severityTrichostatin A increases SMN expression and survival in a mouse model of spinal muscular atrophy.The SMN complex: an assembly machine for RNPsPhase II open label study of valproic acid in spinal muscular atrophyChondrolectin affects cell survival and neuronal outgrowth in in vitro and in vivo models of spinal muscular atrophyU1 snRNP protects pre-mRNAs from premature cleavage and polyadenylationRNA and diseaseEffects of 2,4-diaminoquinazoline derivatives on SMN expression and phenotype in a mouse model for spinal muscular atrophyA novel cell immunoassay to measure survival of motor neurons protein in blood cellsRibonucleoprotein assembly defects correlate with spinal muscular atrophy severity and preferentially affect a subset of spliceosomal snRNPsInactivation of the SMN complex by oxidative stressSpinal Muscular Atrophy: From Defective Chaperoning of snRNP Assembly to Neuromuscular Dysfunction.Spinal muscular atrophy: why do low levels of survival motor neuron protein make motor neurons sick?SMN requirement for synaptic vesicle, active zone and microtubule postnatal organization in motor nerve terminalsQuality control of assembly-defective U1 snRNAs by decapping and 5'-to-3' exonucleolytic digestion.Assays for the identification and prioritization of drug candidates for spinal muscular atrophyTranscriptome profiling of spinal muscular atrophy motor neurons derived from mouse embryonic stem cellsGEMINs: potential therapeutic targets for spinal muscular atrophy?Splicing of a critical exon of human Survival Motor Neuron is regulated by a unique silencer element located in the last intron.RNA exodus to Israel: RNA controlling function in the far reaches of the neuron. Workshop on RNA control on neuronal function.Abnormal interaction of motor neuropathy-associated mutant HspB8 (Hsp22) forms with the RNA helicase Ddx20 (gemin3).Spinal muscular atrophy: diagnosis and management in a new therapeutic era
P2860
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P2860
The survival of motor neurons protein determines the capacity for snRNP assembly: biochemical deficiency in spinal muscular atrophy.
description
2005 nî lūn-bûn
@nan
2005 թուականի Յուլիսին հրատարակուած գիտական յօդուած
@hyw
2005 թվականի հուլիսին հրատարակված գիտական հոդված
@hy
2005年の論文
@ja
2005年論文
@yue
2005年論文
@zh-hant
2005年論文
@zh-hk
2005年論文
@zh-mo
2005年論文
@zh-tw
2005年论文
@wuu
name
The survival of motor neurons ...... cy in spinal muscular atrophy.
@ast
The survival of motor neurons ...... cy in spinal muscular atrophy.
@en
type
label
The survival of motor neurons ...... cy in spinal muscular atrophy.
@ast
The survival of motor neurons ...... cy in spinal muscular atrophy.
@en
prefLabel
The survival of motor neurons ...... cy in spinal muscular atrophy.
@ast
The survival of motor neurons ...... cy in spinal muscular atrophy.
@en
P2093
P2860
P1476
The survival of motor neurons ...... cy in spinal muscular atrophy.
@en
P2093
Amelie K Gubitz
Daniel J Battle
Gideon Dreyfuss
Jeongsik Yong
Stephen J Kolb
P2860
P304
P356
10.1128/MCB.25.13.5543-5551.2005
P407
P577
2005-07-01T00:00:00Z