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Titolo:
IDENTIFICATION OF A (CUG)(N) TRIPLET REPEAT RNA-BINDING PROTEIN AND ITS EXPRESSION IN MYOTONIC-DYSTROPHY
Autore:
TIMCHENKO LT; MILLER JW; TIMCHENKO NA; DEVORE DR; DATAR KV; LIN LJ; ROBERTS R; CASKEY CT; SWANSON MS;
Indirizzi:
BAYLOR COLL MED,CARDIOL SECT,DEPT MED HOUSTON TX 77030 BAYLOR COLL MED,CARDIOL SECT,DEPT MED HOUSTON TX 77030 UNIV FLORIDA,COLL MED,DEPT MOL GENET & MICROBIOL GAINESVILLE FL 32610 UNIV FLORIDA,COLL MED,CTR MAMMALIAN GENET GAINESVILLE FL 32610 BAYLOR COLL MED,DEPT PATHOL HOUSTON TX 77030 MERCK SHARP & DOHME LTD,RES LABS W POINT PA 19486
Titolo Testata:
Nucleic acids research
fascicolo: 22, volume: 24, anno: 1996,
pagine: 4407 - 4414
SICI:
0305-1048(1996)24:22<4407:IOA(TR>2.0.ZU;2-9
Fonte:
ISI
Lingua:
ENG
Soggetto:
CTG REPEAT; TRINUCLEOTIDE REPEAT; ATTENUATION PROTEIN; MESSENGER-RNA; BACILLUS-SUBTILIS; NERVOUS-SYSTEM; HNRNP-I; GENE; TRANSCRIPTS; KINASE;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
45
Recensione:
Indirizzi per estratti:
Citazione:
L.T. Timchenko et al., "IDENTIFICATION OF A (CUG)(N) TRIPLET REPEAT RNA-BINDING PROTEIN AND ITS EXPRESSION IN MYOTONIC-DYSTROPHY", Nucleic acids research, 24(22), 1996, pp. 4407-4414

Abstract

Myotonic dystrophy (DM) is an autosomal dominant neuromuscular disease that is associated with a (CTG)(n) repeat expansion in the 3'-untranslated region of the myotonin protein kinase (Mt-PK) gene. This study reports the isolation and characterization of a (CUG)(n) triplet repeat pre-mRNA/mRNA binding protein that may play an important role in DM pathogenesis. Two HeLa cell proteins, CUG-BP1 and CUG-BP2, have been purified based upon their ability to bind specifically to (CUG)(8) oligonucleotides in vitro, While CUG-BP1 is the major (CUG)(8)-binding activity in normal cells, nuclear CUG-BP2 binding activity increases in DM cells, Both CUG-BP1 and CUG-BP2 have been identified as isoforms of a novel heterogeneous nuclear ribonucleoprotein (hnRNP), hNab50, The CUG-BP/hNab50 protein is localized predominantly in the nucleus and is associated with polyadenylated RNAs in vivo. In vitro RNA-binding/photocrosslinking studies demonstrate that CUG-BP/hNab50 binds to RNAs containing the Mt-PK 3'-UTR, We propose that the (CUG)(n) repeat region in Mt-PK mRNA is a binding site for CUG-BP/hNab50 in vivo, and triplet repeat expansion leads to sequestration of this hnRNP on mutant Mt-PK transcripts.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 23/09/20 alle ore 09:35:53