Nuclear accumulation of mRNAs underlies G4C2-repeat-induced translational repression in a cellular model of C9orf72 ALS.
about
Bidirectional nucleolar dysfunction in C9orf72 frontotemporal lobar degenerationStructural basis of nucleic-acid recognition and double-strand unwinding by the essential neuronal protein Pur-alphaTDP-43 and FUS en route from the nucleus to the cytoplasm.GGGGCC microsatellite RNA is neuritically localized, induces branching defects, and perturbs transport granule functionRetention of hexanucleotide repeat-containing intron in C9orf72 mRNA: implications for the pathogenesis of ALS/FTDGain of Toxicity from ALS/FTD-Linked Repeat Expansions in C9ORF72 Is Alleviated by Antisense Oligonucleotides Targeting GGGGCC-Containing RNAsPoly-dipeptides encoded by the C9ORF72 repeats block global protein translationIdentifying proteins that bind to specific RNAs - focus on simple repeat expansion diseases.Marked Differences in C9orf72 Methylation Status and Isoform Expression between C9/ALS Human Embryonic and Induced Pluripotent Stem Cells.Structural Characteristics of Simple RNA Repeats Associated with Disease and their Deleterious Protein Interactions.Old versus New Mechanisms in the Pathogenesis of ALS.The small heat shock protein B8 (HSPB8) efficiently removes aggregating species of dipeptides produced in C9ORF72-related neurodegenerative diseases.Insights into the pathogenic mechanisms of Chromosome 9 open reading frame 72 (C9orf72) repeat expansions.ALS: Recent Developments from Genetics Studies.Nuclear trafficking in amyotrophic lateral sclerosis and frontotemporal lobar degeneration.Pathogenic determinants and mechanisms of ALS/FTD linked to hexanucleotide repeat expansions in the C9orf72 gene.A short conserved motif in ALYREF directs cap- and EJC-dependent assembly of export complexes on spliced mRNAsThe Role of Dipeptide Repeats in C9ORF72-Related ALS-FTD.Control of mRNA Translation in ALS ProteinopathyEnergy Homeostasis and Abnormal RNA Metabolism in Amyotrophic Lateral SclerosisThe C9ORF72 GGGGCC expansion forms RNA G-quadruplex inclusions and sequesters hnRNP H to disrupt splicing in ALS brains.RNA Misprocessing in C9orf72-Linked Neurodegeneration.In-depth clinico-pathological examination of RNA foci in a large cohort of C9ORF72 expansion carriers.Failure to Deliver and Translate-New Insights into RNA Dysregulation in ALS.PURA, the gene encoding Pur-alpha, member of an ancient nucleic acid-binding protein family with mammalian neurological functions.Sense-encoded poly-GR dipeptide repeat proteins correlate to neurodegeneration and uniquely co-localize with TDP-43 in dendrites of repeat-expanded C9orf72 amyotrophic lateral sclerosis.RAN translation at C9orf72-associated repeat expansions is selectively enhanced by the integrated stress response.The roles of intrinsic disorder-based liquid-liquid phase transitions in the "Dr. Jekyll-Mr. Hyde" behavior of proteins involved in amyotrophic lateral sclerosis and frontotemporal lobar degeneration.Dysregulation of chromatin remodelling complexes in amyotrophic lateral sclerosis.Dysregulated molecular pathways in amyotrophic lateral sclerosis-frontotemporal dementia spectrum disorder.Unraveling the Role of RNA Mediated Toxicity of C9orf72 Repeats in C9-FTD/ALS.mRNP assembly, axonal transport, and local translation in neurodegenerative diseases.Assay to Measure Nucleocytoplasmic Transport in Real Time within Motor Neuron-like NSC-34 Cells.Perturbed autophagy and DNA repair converge to promote neurodegeneration in amyotrophic lateral sclerosis and dementia.Accumulation of poly(A) RNA in nuclear granules enriched in Sam68 in motor neurons from the SMNΔ7 mouse model of SMA.
P2860
Q33577239-E2AE4470-7E75-40B7-B545-E1D77FCB2DCEQ34508071-A979CE67-B2BD-49EE-8D5C-AF3C54353334Q36336278-7D74BCF8-D566-4CFD-A7B2-6DEAAA02F044Q36594209-7113BE1F-54BB-4775-895B-9462152534D7Q36616570-E2695E55-3FFE-46CA-A08C-A6A8C93F6B5AQ36880235-2660BDD8-78DA-4C6D-BFA1-56CFEFE9064DQ37178589-7CB1A116-EF11-4774-A892-D9D68C45A8C8Q37401085-5179F821-BD4E-4844-A8F5-03250E240215Q37410193-271968CF-0D0E-401E-8C37-33FEE37599A5Q37747172-42C3C179-C6A4-4A7D-8141-BC609846581DQ38700722-82BC80CC-83B5-4A5D-AC87-960C0FBCBF53Q38728380-A1037939-7D24-4384-BB6F-3DC3DE654E40Q38789426-512F0720-0DFB-462B-95E5-9E23B7C3183DQ38817325-90B3FFBC-31D3-48E6-9D0C-D0E8476EE43BQ38920545-741549C8-58E7-4043-BC47-2C59C825B456Q38952327-45BF5DD5-0B97-4DCD-BFD6-3218F4705413Q39142495-B73755F6-82D9-4A33-A3EE-44E9AC2A9954Q39153670-791964F4-C21C-40D7-8859-1639D58C00E3Q39225382-15FAA46E-B912-4643-A1A2-98264EC381EDQ39316849-069FE6DB-F211-4E77-8BEA-D46C815CBDDFQ39396328-53896402-44FA-4146-A451-3C230A612A59Q39458329-C563F803-694E-41BE-AD3A-7DF0BE7B155DQ40999782-50A3CE89-6D57-49DB-A868-1FCF95FFE536Q41458537-D572FA68-9AE5-45BA-9E2C-EE609FDFDF17Q46703061-9B62D788-5A63-426F-8FE6-3A230A2E43CDQ46799396-84D41509-6FDD-4C4E-A713-5F3ACBF8F363Q47313248-FBFCF024-EE0A-4A4D-B5E2-EB81095FA2FDQ47693846-E96FBC54-4DCB-45FE-B2DA-08BB17367438Q47707307-4C1B4DD2-9BB7-4F0B-B1EA-DD8E54EC9759Q47946104-BE561552-6E81-4DE2-A285-ABD8983B709EQ49314473-84F6DC2B-205E-4F30-A965-BE8930DF12A0Q49808601-3B92C368-3D41-480E-B957-931256101895Q50200695-33BAF445-25C8-4182-836E-B1D8DE8594D5Q52627910-1F37C029-D24A-4EAF-98D2-871BE274C792Q55511978-398B506C-F546-4C61-B20E-7F8EFBD4EA8D
P2860
Nuclear accumulation of mRNAs underlies G4C2-repeat-induced translational repression in a cellular model of C9orf72 ALS.
description
2015 nî lūn-bûn
@nan
2015 թուականի Մարտին հրատարակուած գիտական յօդուած
@hyw
2015 թվականի մարտին հրատարակված գիտական հոդված
@hy
2015年の論文
@ja
2015年論文
@yue
2015年論文
@zh-hant
2015年論文
@zh-hk
2015年論文
@zh-mo
2015年論文
@zh-tw
2015年论文
@wuu
name
Nuclear accumulation of mRNAs ...... cellular model of C9orf72 ALS.
@ast
Nuclear accumulation of mRNAs ...... cellular model of C9orf72 ALS.
@en
Nuclear accumulation of mRNAs ...... cellular model of C9orf72 ALS.
@nl
type
label
Nuclear accumulation of mRNAs ...... cellular model of C9orf72 ALS.
@ast
Nuclear accumulation of mRNAs ...... cellular model of C9orf72 ALS.
@en
Nuclear accumulation of mRNAs ...... cellular model of C9orf72 ALS.
@nl
prefLabel
Nuclear accumulation of mRNAs ...... cellular model of C9orf72 ALS.
@ast
Nuclear accumulation of mRNAs ...... cellular model of C9orf72 ALS.
@en
Nuclear accumulation of mRNAs ...... cellular model of C9orf72 ALS.
@nl
P2093
P50
P356
P1476
Nuclear accumulation of mRNAs ...... cellular model of C9orf72 ALS.
@en
P2093
Alessandra Giorgi
Alessia Serrano
Claudia Bagni
Francesca Bozzo
Gianluca Cestra
Maria Eugenia Schininà
Monica Nencini
Simona Rossi
Tilmann Achsel
Valeria Gerbino
P304
P356
10.1242/JCS.165332
P407
P577
2015-03-18T00:00:00Z