Htra2-beta 1 stimulates an exonic splicing enhancer and can restore full-length SMN expression to survival motor neuron 2 (SMN2)
about
Distinct sequence motifs within the 68-kDa subunit of cleavage factor Im mediate RNA binding, protein-protein interactions, and subcellular localizationThe splicing regulator Sam68 binds to a novel exonic splicing silencer and functions in SMN2 alternative splicing in spinal muscular atrophyHeterogeneous nuclear ribonucleoprotein G regulates splice site selection by binding to CC(A/C)-rich regions in pre-mRNATissue-specific reduction in splicing efficiency of IKBKAP due to the major mutation associated with familial dysautonomia.Human RNPS1 and its associated factors: a versatile alternative pre-mRNA splicing regulator in vivoA positive modifier of spinal muscular atrophy in the SMN2 geneThe RNA binding protein hnRNP Q modulates the utilization of exon 7 in the survival motor neuron 2 (SMN2) geneTDP-43 overexpression enhances exon 7 inclusion during the survival of motor neuron pre-mRNA splicingDifferential 3' splice site recognition of SMN1 and SMN2 transcripts by U2AF and U2 snRNPThe RNA Splicing Response to DNA DamageSpinal muscular atrophy: from gene discovery to clinical trialsApplicability of histone deacetylase inhibition for the treatment of spinal muscular atrophySplicing therapy for neuromuscular diseaseHigh expression level of Tra2-β1 is responsible for increased SMN2 exon 7 inclusion in the testis of SMA miceNeuronal-specific deficiency of the splicing factor Tra2b causes apoptosis in neurogenic areas of the developing mouse brainMolecular basis of purine-rich RNA recognition by the human SR-like protein Tra2-β1Characterization of the RNA recognition mode of hnRNP G extends its role in SMN2 splicing regulationAn in vivo reporter system for measuring increased inclusion of exon 7 in SMN2 mRNA: potential therapy of SMAA short antisense oligonucleotide masking a unique intronic motif prevents skipping of a critical exon in spinal muscular atrophySAHA ameliorates the SMA phenotype in two mouse models for spinal muscular atrophyTau exons 2 and 10, which are misregulated in neurodegenerative diseases, are partly regulated by silencers which bind a SRp30c.SRp55 complex that either recruits or antagonizes htra2beta1Splicing factor transformer-2β (Tra2β) regulates the expression of regulator of G protein signaling 4 (RGS4) gene and is induced by morphineIdentification of evolutionarily conserved exons as regulated targets for the splicing activator tra2β in developmentSMN in spinal muscular atrophy and snRNP biogenesisSite identification in high-throughput RNA-protein interaction dataPathological impact of SMN2 mis-splicing in adult SMA miceSubtle mutations in the SMN1 gene in Chinese patients with SMA: p.Arg288Met mutation causing SMN1 transcript exclusion of exon7.Enhancement of SMN2 exon 7 inclusion by antisense oligonucleotides targeting the exon.Prediction of alternatively skipped exons and splicing enhancers from exon junction arrays.Mouse survival motor neuron alleles that mimic SMN2 splicing and are inducible rescue embryonic lethality early in development but not lateSpinal muscular atrophy: mechanisms and therapeutic strategies.Treatment of spinal muscular atrophy by sodium butyrate.Intron 7 conserved sequence elements regulate the splicing of the SMN genes.Splicing of the Survival Motor Neuron genes and implications for treatment of SMAIdentification of novel compounds that increase SMN protein levels using an improved SMN2 reporter cell assay.A negatively acting bifunctional RNA increases survival motor neuron both in vitro and in vivo.TIA1 prevents skipping of a critical exon associated with spinal muscular atrophy.Restoration of the cystic fibrosis transmembrane conductance regulator function by splicing modulation.A humanized Smn gene containing the SMN2 nucleotide alteration in exon 7 mimics SMN2 splicing and the SMA disease phenotype.SMN-inducing compounds for the treatment of spinal muscular atrophy.
P2860
Q24294619-60BDDEFF-9EA4-4700-A169-A490F32631C3Q24301061-8593E62A-3596-4D25-8A4F-7C9B3EA75978Q24321263-F9680FBB-18E5-4B5C-A3D8-87C33D09D271Q24531976-BAC67ABD-6A7D-475D-8A5E-1EF906A55217Q24629384-51924123-3AF0-4163-9E3F-42C9CCC7B1D9Q24646149-88C7362A-C28F-4CC2-B1F9-45067A380BE7Q24648050-6CFEE8BB-DD38-4686-A13F-C17EAC3291B9Q24651520-31D97B2E-B5BF-4065-B7A0-EF40C35C456CQ24651863-38EED3F5-406A-4311-B3DE-B29F3BAD975BQ26777046-426B39CD-B3A1-4103-A38D-097052C2CDC1Q26824894-ED7020AA-36AC-4149-AF01-5A6E4CA10046Q26864605-07113DBE-87EB-4836-95FD-6BB8C53D5B77Q27009581-F8C7213A-37A0-449A-9A30-EF95AD50D3A6Q27311317-0798F9A6-04CA-4931-A010-25F6B04BC4C6Q27321571-EEF9E217-9639-4DB7-AE19-2285F57AE991Q27666966-E4F6C25A-59D1-4640-939C-44E6BA83D95EQ27682769-346CFBAC-BB3E-4A48-90B7-3B955848DECFQ28204816-634E7FA9-E63B-4CC9-99F5-8DBDFE8E4614Q28244480-250D6D11-C854-422C-A666-50275A4DA3B3Q28271235-9CA865AE-AA4A-493A-8497-024CDEF62A76Q28306183-AD78B32C-0501-4D7F-AFA3-7DDBBBBC05F2Q28535422-8F0BCD7B-4E0A-4367-8624-23FC808841DFQ28588811-47D3FB39-F21C-48DC-84AF-C355404D14C7Q29013847-5DD28AF9-35D5-45F6-AE3C-7E60B1906E1BQ30528777-FADCD4FE-9614-441E-8F5E-A1C61E82CFC9Q30551534-3C5A8942-9703-4E57-845E-18D5BCA78687Q31096071-7F5B87FB-12B2-4BA4-B22F-71BC16CECA3EQ33278100-1BF446B8-8975-46BE-B1AC-1B3B83D9885DQ33385851-ABC3E26B-0E15-46B7-A4AD-A34DBF9C0B8BQ33797677-2DC36894-EBFB-48F9-B551-2D54622E7ED2Q33871311-A2221BFA-BFD3-4144-AC61-AE72A93395D6Q33933841-BE36C918-36ED-4FE1-B2C0-C5D559D6741BQ33937593-E4F9861E-8BCD-412E-858E-FAFC658A91CBQ34065993-768CD8A5-AF02-4DAB-86DC-E6F641F44A9AQ34123129-B73BAE65-AD99-49B5-85E6-B3A8CD056959Q34132150-C17B0C2A-2414-4939-A60F-09A32101503AQ34156826-CD5119CB-E59A-451B-B020-B75332B971B3Q34166299-04E6E0DC-BD2A-4BAD-830B-6DC500540A92Q34184793-1F715293-1442-4EF1-B33D-9360DE639032Q34312156-617267A1-3E68-4AD8-8E33-A3D0A9D6885E
P2860
Htra2-beta 1 stimulates an exonic splicing enhancer and can restore full-length SMN expression to survival motor neuron 2 (SMN2)
description
2000 nî lūn-bûn
@nan
2000 թուականի Օգոստոսին հրատարակուած գիտական յօդուած
@hyw
2000 թվականի օգոստոսին հրատարակված գիտական հոդված
@hy
2000年の論文
@ja
2000年論文
@yue
2000年論文
@zh-hant
2000年論文
@zh-hk
2000年論文
@zh-mo
2000年論文
@zh-tw
2000年论文
@wuu
name
Htra2-beta 1 stimulates an exo ...... survival motor neuron 2 (SMN2)
@ast
Htra2-beta 1 stimulates an exo ...... survival motor neuron 2 (SMN2)
@en
type
label
Htra2-beta 1 stimulates an exo ...... survival motor neuron 2 (SMN2)
@ast
Htra2-beta 1 stimulates an exo ...... survival motor neuron 2 (SMN2)
@en
prefLabel
Htra2-beta 1 stimulates an exo ...... survival motor neuron 2 (SMN2)
@ast
Htra2-beta 1 stimulates an exo ...... survival motor neuron 2 (SMN2)
@en
P2093
P2860
P356
P1476
Htra2-beta 1 stimulates an exo ...... survival motor neuron 2 (SMN2)
@en
P2093
P2860
P304
P356
10.1073/PNAS.160181697
P407
P577
2000-08-01T00:00:00Z