Ribonucleoprotein assembly defects correlate with spinal muscular atrophy severity and preferentially affect a subset of spliceosomal snRNPs
about
Development of a single vector system that enhances trans-splicing of SMN2 transcriptsRestoration of full-length SMN promoted by adenoviral vectors expressing RNA antisense oligonucleotides embedded in U7 snRNAsAlternative splicing events are a late feature of pathology in a mouse model of spinal muscular atrophySpinal muscular atrophyModeling spinal muscular atrophy in DrosophilaThe zinc finger protein ZPR1 is a potential modifier of spinal muscular atrophy.The contribution of mouse models to understanding the pathogenesis of spinal muscular atrophyA SMN missense mutation complements SMN2 restoring snRNPs and rescuing SMA miceSpinal muscular atrophy: development and implementation of potential treatmentsSplicing therapy for neuromuscular diseaseSolution structure of the core SMN–Gemin2 complexA short antisense oligonucleotide masking a unique intronic motif prevents skipping of a critical exon in spinal muscular atrophyA degron created by SMN2 exon 7 skipping is a principal contributor to spinal muscular atrophy severityThe significant other: splicing by the minor spliceosomeRescue of a severe mouse model for spinal muscular atrophy by U7 snRNA-mediated splicing modulationRegulation of SMN protein stabilityChondrolectin affects cell survival and neuronal outgrowth in in vitro and in vivo models of spinal muscular atrophyA role for SMN exon 7 splicing in the selective vulnerability of motor neurons in spinal muscular atrophySMN in spinal muscular atrophy and snRNP biogenesisU1 snRNP protects pre-mRNAs from premature cleavage and polyadenylationMechanisms of alternative splicing regulation: insights from molecular and genomics approachesRNA and diseaseEffects of 2,4-diaminoquinazoline derivatives on SMN expression and phenotype in a mouse model for spinal muscular atrophyCNS-targeted gene therapy improves survival and motor function in a mouse model of spinal muscular atrophy.The COPI vesicle complex binds and moves with survival motor neuron within axons.Postsymptomatic restoration of SMN rescues the disease phenotype in a mouse model of severe spinal muscular atrophy.Protective effects of butyrate-based compounds on a mouse model for spinal muscular atrophy.Pharmacokinetics, pharmacodynamics, and efficacy of a small-molecule SMN2 splicing modifier in mouse models of spinal muscular atrophySmall nuclear RNAs and mRNAs: linking RNA processing and transport to spinal muscular atrophy.TDP-43 expression in mouse models of amyotrophic lateral sclerosis and spinal muscular atrophy.Functional interaction between FUS and SMN underlies SMA-like splicing changes in wild-type hFUS mice.Trans-splicing-mediated improvement in a severe mouse model of spinal muscular atrophy.A novel functional role for MMSET in RNA processing based on the link between the REIIBP isoform and its interaction with the SMN complexSpinal 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?Mouse survival motor neuron alleles that mimic SMN2 splicing and are inducible rescue embryonic lethality early in development but not lateSpinal muscular atrophy: new and emerging insights from model miceProteomic assessment of a cell model of spinal muscular atrophyThe Survival of Motor Neuron Protein Acts as a Molecular Chaperone for mRNP AssemblySpecific splicing defects in S. pombe carrying a degron allele of the Survival of Motor Neuron gene
P2860
Q21092173-769F61A6-4F3B-4A32-8F39-4E438876CC8CQ21142695-6833B30A-7130-48AC-AB60-DC1F967E0FAFQ21144988-4C0B96D7-403F-453D-A829-CE73A5A9AED9Q21202863-193E8A99-6121-4D9F-AE4A-F9E154DF019AQ21562542-44D3B083-1018-49E3-B214-0E7D7299E474Q24307665-75ED1E16-51D5-40DD-9642-9FEB433532DBQ24604444-F33E8B3C-E71F-46DC-8F8F-8E9B6CB8B6C8Q24657021-932A3D51-B2CA-4285-8480-6FBCCAF0D473Q26861377-70343A68-D6D5-451D-95F9-548B392961A9Q27009581-26E832DD-EB62-4DC6-BB11-55B98D42D328Q27679173-DD80091D-94FE-40C1-B752-B24508418E8CQ28244480-39429F40-83AC-456F-9630-68A8EFB2DE96Q28274685-C1D0E9E4-5888-4501-A2AE-AB1C9EEC6259Q28277280-2D77BB87-12A6-46A4-9F0F-B2C5899217B7Q28300972-AE1960F0-F52A-4DCE-A3F4-1673193938A2Q28304878-9C0E277C-EA40-4968-B95B-1491D526CB61Q28506113-A265AA77-AA75-4897-BA3B-BF752D6C8310Q28586960-491D279F-40F3-49F4-BC0C-3498F8F07242Q29013847-13888EB3-75A5-45BB-9F6F-BE400B0BBC53Q29032058-9A1BF038-E0ED-441D-AFA6-69E7E5137100Q29615084-7AE87821-5302-4BA6-826A-D3300615AC87Q29615183-D3C47704-A186-4252-B589-B1587DA450CCQ30492560-AD6E570C-F124-467A-8EE8-561AB2C73116Q30493891-972AD49A-165C-449F-A88B-6CDAA827C199Q30499358-5AB57253-2E66-455A-9376-9F135F4895F3Q30503157-25D8F0DC-DB56-448B-89DC-86B36D5E2B0AQ30743508-E2F68DEF-5510-4455-973A-29C39269F1F6Q30821687-E42CF958-090E-430A-BABA-B62CDCF81BE5Q33356209-631F54B2-A393-450E-8EED-B2CEBBA72B55Q33379948-9D47ECBB-92B0-4AF3-8962-3A09303C8EC4Q33700562-6BD84D4C-AE43-4B2C-8B28-4D8CF4C542CAQ33720730-6AD30DF0-F72E-403A-A0A7-68BA6C389FD6Q33748763-2294BE80-E985-4F39-81B5-5F52508E5AB9Q33775770-8C107496-00DE-4EED-90AA-F6CB9CB821ABQ33785933-BB0A270C-101A-4C1A-B7F8-C368AD2C9F83Q33797677-B3326D59-60CC-445E-B16E-1EB0BD77D5B1Q33819144-745BAB0C-9D6E-48B9-98EA-806EDF0F3C4DQ33840295-5204B402-1DEA-45DB-A0C0-666256304583Q33858798-9AAE0187-EAD9-4FD7-A6A5-223CB797594BQ33914362-F0D02029-B3F4-4673-B52A-3E1DCA4EB1DB
P2860
Ribonucleoprotein assembly defects correlate with spinal muscular atrophy severity and preferentially affect a subset of spliceosomal snRNPs
description
2007 nî lūn-bûn
@nan
2007 թուականի Սեպտեմբերին հրատարակուած գիտական յօդուած
@hyw
2007 թվականի սեպտեմբերին հրատարակված գիտական հոդված
@hy
2007年の論文
@ja
2007年論文
@yue
2007年論文
@zh-hant
2007年論文
@zh-hk
2007年論文
@zh-mo
2007年論文
@zh-tw
2007年论文
@wuu
name
Ribonucleoprotein assembly def ...... subset of spliceosomal snRNPs
@ast
Ribonucleoprotein assembly def ...... subset of spliceosomal snRNPs
@en
Ribonucleoprotein assembly def ...... subset of spliceosomal snRNPs.
@nl
type
label
Ribonucleoprotein assembly def ...... subset of spliceosomal snRNPs
@ast
Ribonucleoprotein assembly def ...... subset of spliceosomal snRNPs
@en
Ribonucleoprotein assembly def ...... subset of spliceosomal snRNPs.
@nl
prefLabel
Ribonucleoprotein assembly def ...... subset of spliceosomal snRNPs
@ast
Ribonucleoprotein assembly def ...... subset of spliceosomal snRNPs
@en
Ribonucleoprotein assembly def ...... subset of spliceosomal snRNPs.
@nl
P2093
P2860
P50
P921
P1433
P1476
Ribonucleoprotein assembly def ...... subset of spliceosomal snRNPs
@en
P2093
Francesca Gabanella
Luciano Saieva
Matthew E R Butchbach
P2860
P356
10.1371/JOURNAL.PONE.0000921
P407
P577
2007-09-26T00:00:00Z