Nuclear poly(A)-binding protein aggregates misplace a pre-mRNA outside of SC35 speckle causing its abnormal splicing.
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PABPN1 gene therapy for oculopharyngeal muscular dystrophyNuclear speckles: molecular organization, biological function and role in disease.An alanine expanded PABPN1 causes increased utilization of intronic polyadenylation sites.Nuclear poly(A) binding protein 1 (PABPN1) and Matrin3 interact in muscle cells and regulate RNA processing.Functional impact of an oculopharyngeal muscular dystrophy mutation in PABPN1.Novel mouse models of oculopharyngeal muscular dystrophy (OPMD) reveal early onset mitochondrial defects and suggest loss of PABPN1 may contribute to pathology.Accumulation of poly(A) RNA in nuclear granules enriched in Sam68 in motor neurons from the SMNΔ7 mouse model of SMA.Post-transcriptional regulation of Pabpn1 by the RNA binding protein HuR
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P2860
Nuclear poly(A)-binding protein aggregates misplace a pre-mRNA outside of SC35 speckle causing its abnormal splicing.
description
article científic
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article scientifique
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articolo scientifico
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artigo científico
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bilimsel makale
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scientific article published on 09 August 2016
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vedecký článok
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vetenskaplig artikel
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videnskabelig artikel
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vědecký článek
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name
Nuclear poly
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Nuclear poly(A)-binding protei ...... causing its abnormal splicing.
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type
label
Nuclear poly
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Nuclear poly(A)-binding protei ...... causing its abnormal splicing.
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prefLabel
Nuclear poly
@nl
Nuclear poly(A)-binding protei ...... causing its abnormal splicing.
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P2093
P2860
P50
P356
P1476
Nuclear poly(A)-binding protei ...... causing its abnormal splicing.
@en
P2093
Alberto Malerba
Fanny Roth
George Dickson
Jean Lacau St Guily
Linda Popplewell
Martine Oloko
Michael N Antoniou
Pierre Klein
Pierre de la Grange
Susan Jarmin
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P304
10929-10945
P356
10.1093/NAR/GKW703
P407
P577
2016-08-09T00:00:00Z