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Titolo:
A NUCLEAR ROLE FOR THE FRAGILE-X MENTAL-RETARDATION PROTEIN
Autore:
FRIDELL RA; BENSON RE; HUA J; BOGERD HP; CULLEN BR;
Indirizzi:
DUKE UNIV,MED CTR,HOWARD HUGHES MED INST,ROOM 426,CARL BLDG,RES DR DURHAM NC 27710 DUKE UNIV,MED CTR,DEPT GENET DURHAM NC 27710
Titolo Testata:
EMBO journal
fascicolo: 19, volume: 15, anno: 1996,
pagine: 5408 - 5414
SICI:
0261-4189(1996)15:19<5408:ANRFTF>2.0.ZU;2-T
Fonte:
ISI
Lingua:
ENG
Soggetto:
REV ACTIVATION DOMAIN; VIRAL MESSENGER-RNA; VIRUS TYPE-1 REV; TRANS-ACTIVATOR; CGG-REPEAT; GENE; FMR-1; IDENTIFICATION; BINDING; EXPORT;
Keywords:
FRAGILE X SYNDROME; HIV-1; NUCLEAR EXPORT; RNA BINDING;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
39
Recensione:
Indirizzi per estratti:
Citazione:
R.A. Fridell et al., "A NUCLEAR ROLE FOR THE FRAGILE-X MENTAL-RETARDATION PROTEIN", EMBO journal, 15(19), 1996, pp. 5408-5414

Abstract

Fragile X syndrome results from lack of expression of a functional form of Fragile X mental retardation protein (FMRP), a cytoplasmic RNA-binding protein of uncertain function, Here, we report that FMRP contains a nuclear export signal (NES) that is similar to the NES recently identified in the Rev regulatory protein of human immunodeficiency virus type 1 (HIV-1). Mutation of this FMRP NES results in mislocalizationof FMRP to the cell nucleus, The FMRP NES is encoded within exon 14 of the FMR1 gene, thus explaining the aberrant nuclear localization of a natural isoform of FMRP that lacks this exon, The NES of FMRP can substitute fully for the Rev NES in mediating Rev-dependent nuclear RNA export and specifically binds a nucleoporin-like cellular cofactor that has been shown to mediate Rev NES function, Together, these findingsdemonstrate that the normal function of FMRP involves entry into the nucleus followed by export via a pathway that is identical to the one utilized by HIV-1 Rev, In addition, these data raise the possibility that FMRP could play a role in mediating the nuclear export of its currently undefined cellular RNA target(s).

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 29/09/20 alle ore 04:37:15