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Titolo:
Rescue of a telomere length defect of Nijmegen breakage syndrome cells requires NBS and telomerase catalytic subunit
Autore:
Ranganathan, V; Heine, WF; Ciccone, DN; Rudolph, KL; Wu, XH; Chang, S; Hai, H; Ahearn, IM; Livingston, DM; Resnick, I; Rosen, F; Seemanova, E; Jarolim, P; DePinho, RA; Weaver, DT;
Indirizzi:
Ctr Blood Res, Boston, MA 02115 USA Ctr Blood Res Boston MA USA 02115Ctr Blood Res, Boston, MA 02115 USA Harvard Univ, Sch Med, Dept Microbiol & Mol Genet, Boston, MA 02115 USA Harvard Univ Boston MA USA 02115 robiol & Mol Genet, Boston, MA 02115 USA Dana Farber Canc Inst, Boston, MA 02115 USA Dana Farber Canc Inst Boston MA USA 02115 Canc Inst, Boston, MA 02115 USA Harvard Univ, Sch Med, Dept Med & Genet, Boston, MA 02115 USA Harvard Univ Boston MA USA 02115 , Dept Med & Genet, Boston, MA 02115 USA Res Inst Pediat Hematol, Dept Clin Immunol, Moscow, Russia Res Inst PediatHematol Moscow Russia Dept Clin Immunol, Moscow, Russia Charles Univ, Sch Med, Dept Med Genet, Prague, Czech Republic Charles Univ Prague Czech Republic pt Med Genet, Prague, Czech Republic Inst Hematol & Blood Transfus, CR-12820 Prague, Czech Republic Inst Hematol & Blood Transfus Prague Czech Republic CR-12820 ch Republic Brigham & Womens Hosp, Dept Pathol, Boston, MA 02115 USA Brigham & Womens Hosp Boston MA USA 02115 pt Pathol, Boston, MA 02115 USA
Titolo Testata:
CURRENT BIOLOGY
fascicolo: 12, volume: 11, anno: 2001,
pagine: 962 - 966
SICI:
0960-9822(20010626)11:12<962:ROATLD>2.0.ZU;2-B
Fonte:
ISI
Lingua:
ENG
Soggetto:
DNA-DAMAGE RESPONSE; ATAXIA-TELANGIECTASIA; SACCHAROMYCES-CEREVISIAE; HUMAN FIBROBLASTS; REPAIR PROTEINS; PHOSPHORYLATION; TRF2; P53; INSTABILITY; DISORDER;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
39
Recensione:
Indirizzi per estratti:
Indirizzo: Weaver, DT Ctr Blood Res, 200 Longwood Ave, Boston, MA 02115 USA Ctr BloodRes 200 Longwood Ave Boston MA USA 02115 MA 02115 USA
Citazione:
V. Ranganathan et al., "Rescue of a telomere length defect of Nijmegen breakage syndrome cells requires NBS and telomerase catalytic subunit", CURR BIOL, 11(12), 2001, pp. 962-966

Abstract

Nijmegen breakage syndrome (NBS) is a rare human disease displaying chromosome instability, radiosensitivity, cancer predisposition, immunodeficiency, and other defects [1, 2]. NBS is complexed with MRE11 and RAD50 in a DNA repair complex [3-5] and is localized to telomere ends in association with TRF proteins [6, 7]. We show that blood cells from NBS patients have shortened telomere DNA ends. Likewise, cultured NBS fibroblasts that exhibit a premature growth cessation were observed with correspondingly shortened telomeres. Introduction of the catalytic subunit of telomerase, TERT, was alone sufficient to increase the proliferative capacity of NBS fibroblasts. However, Has, but not TERT, restores the capacity of NBS cells to survive gamma irradiation damage. Strikingly, NBS promotes telomere elongation in conjunction with TERT in NBS fibroblasts. These results suggest that NBS is a required accessory protein for telomere extension. Since NBS patients have shortened telomeres, these defects may contribute to the chromosome instabilityand disease associated with NBS patients. (C) 2001 Elsevier Science Ltd. All rights reserved.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 29/09/20 alle ore 23:55:02