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Titolo:
The fragile X-related proteins FXR1P and FXR2P contain a functional nucleolar-targeting signal equivalent to the HIV-1 regulatory proteins
Autore:
Tamanini, F; Kirkpatrick, LL; Schonkeren, J; van Unen, L; Bontekoe, C; Bakker, C; Nelson, DL; Galjaard, H; Oostra, BA; Hoogeveen, AT;
Indirizzi:
Erasmus Univ, Dept Clin Genet, NL-3000 DR Rotterdam, Netherlands Erasmus Univ Rotterdam Netherlands NL-3000 DR DR Rotterdam, Netherlands Erasmus Univ, Ctr Biomed Genet, NL-3000 DR Rotterdam, Netherlands Erasmus Univ Rotterdam Netherlands NL-3000 DR DR Rotterdam, Netherlands Baylor Coll Med, Dept Mol & Human Genet, Houston, TX 77030 USA Baylor CollMed Houston TX USA 77030 & Human Genet, Houston, TX 77030 USA
Titolo Testata:
HUMAN MOLECULAR GENETICS
fascicolo: 10, volume: 9, anno: 2000,
pagine: 1487 - 1493
SICI:
0964-6906(20000612)9:10<1487:TFXPFA>2.0.ZU;2-O
Fonte:
ISI
Lingua:
ENG
Soggetto:
MENTAL-RETARDATION PROTEIN; SELECTIVE RNA-BINDING; MESSENGER-RNA; NUCLEAR EXPORT; FMR1 PROTEIN; GENE; LOCALIZATION; REV; EXPRESSION; DOMAINS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
55
Recensione:
Indirizzi per estratti:
Indirizzo: Hoogeveen, AT Erasmus Univ, Dept Clin Genet, NL-3000 DR Rotterdam, Netherlands Erasmus Univ Rotterdam Netherlands NL-3000 DR Netherlands
Citazione:
F. Tamanini et al., "The fragile X-related proteins FXR1P and FXR2P contain a functional nucleolar-targeting signal equivalent to the HIV-1 regulatory proteins", HUM MOL GEN, 9(10), 2000, pp. 1487-1493

Abstract

Fragile X syndrome is caused by the absence of the INTRODUCTION fragile X mental-retardation protein (FMRP). FMRP and the fragile X-related proteins 1 and 2 (FXR1P and FXR2P) form a gene family with functional similarities, such as RNA binding, polyribosomal association and nucleocytoplasmic shuttling. In a previous study, we found that FMRP and FXR1P shuttle between cytoplasm and nucleoplasm, while FXR2P shuttles between cytoplasm and nucleolus. The nuclear and nucleolar-targeting properties of these proteins were investigated further. Here, we show that FXR2P contains in its C-terminal part, a stretch of basic amino acids 'RPQRRNRSRRRRFR' that resemble the nucleolar-targeting signal (NoS) of the viral protein Rev. This particular sequence is also present within exon 15 of the FXR1 gene. This exon undergoes alternative splicing and is therefore only present in some of the FXR1P isoforms, We investigated the intracellular distribution of various FXR1P isoformswith (iso-e and iso-f) and without (iso-d) the potential NoS in transfected COS cells treated with the nuclear export inhibitor teptomycin-B. Both iso-e and iso-f showed a nucleolar localization, as observed for FXR2P; iso-dwas detected in the nucleoplasm outside the nucleoli, Further, when a labelled le-residue synthetic peptide corresponding to the NoS of FXR1P was added to human fibroblast cultures a clear nucleolar signal was observed. Based on these data we argue that the intranuclear distribution of FXR2P and FXR1P isoforms is very likely to be mediated by a similar NoS localized in their C-terminal region. This domain is absent in some FXR1P isoforms as wellas in all FMRP isoforms, suggesting functional differences for this familyof proteins, possibly related to RNA metabolism in different tissues.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 02/04/20 alle ore 02:22:47