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Titolo:
NIJMEGEN BREAKAGE SYNDROME CELLS FAIL TO INDUCE THE P53-MEDIATED DNA-DAMAGE RESPONSE FOLLOWING EXPOSURE TO IONIZING-RADIATION
Autore:
WIM JM; VUILLAUME M; CHRZANOWSKA K; SMEETS D; SPERLING K; HALL J;
Indirizzi:
INT AGCY RES CANC,UNIT MECHANISMS CARCINOGENESIS,150 COURS ALBERT THOMAS F-69372 LYON 08 FRANCE INT AGCY RES CANC,UNIT MECHANISMS CARCINOGENESIS F-69372 LYON 08 FRANCE CHILDRENS MEM HLTH INST,DEPT HUMAN GENET PL-04736 WARSAW POLAND UNIV NIJMEGEN HOSP,DEPT HUMAN GENET NL-6500 HB NIJMEGEN NETHERLANDS HUMBOLDT UNIV BERLIN,VIRCHOW KLINIKUM,INST HUMAN GENET D-13353 BERLINGERMANY
Titolo Testata:
Molecular and cellular biology
fascicolo: 9, volume: 17, anno: 1997,
pagine: 5016 - 5022
SICI:
0270-7306(1997)17:9<5016:NBSCFT>2.0.ZU;2-Z
Fonte:
ISI
Lingua:
ENG
Soggetto:
ATAXIA-TELANGIECTASIA GENE; CHROMOSOMAL INSTABILITY DISORDER; HUMAN FIBROBLASTS; GROWTH ARREST; P53; CHECKPOINT; RADIOSENSITIVITY; COMPLEMENTATION; IRRADIATION; PROTEIN;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
42
Recensione:
Indirizzi per estratti:
Citazione:
J.M. Wim et al., "NIJMEGEN BREAKAGE SYNDROME CELLS FAIL TO INDUCE THE P53-MEDIATED DNA-DAMAGE RESPONSE FOLLOWING EXPOSURE TO IONIZING-RADIATION", Molecular and cellular biology, 17(9), 1997, pp. 5016-5022

Abstract

The functionality of the p53-mediated pathway, activated in response to DNA damage, has been assessed in primary fibroblast cell cultures and Epstein-Barr virus-transformed lymphoblastoid cell lines derived from Nijmegen breakage syndrome (NBS) patients, This autosomal recessivedisease is characterized by microcephaly, growth and mental retardation, chromosomal instability, radiosensitivity, and high cancer incidence, The recent mapping of the NBS gene to chromosome 8q21 demonstratesthat NBS is genetically distinct from ataxia telangiectasia (AT), Changes in p53 protein levels were significantly reduced and delayed in all the NBS fibroblast cell cultures and lymphoblastoid cell lines examined compared to normal cultures over a 4-h period postirradiation (5 Gy). The transcriptional activation of p21(WAF1/CIP1) mRNA was also lower in 12 NBS fibroblast cultures examined. In agreement with an abrogated p53 function, NBS cells exposed to ionizing radiation show an abnormal cell cycle arrest at G(1)-S and a prolonged accumulation of cells in the G, phase, In contrast, exposure to the alkylating agent methyl methanesulfonate results in similar increases of p53 and p21(WAF1/CIP1) mRNA in both cell types, The ATM gene transcript was found to be expressed at similar levels in NBS and normal cells, whereas it was strongly reduced in the AT homozygote cells examined, These results suggest that the ATM gene product cannot substitute for that of the NBS gene in the signaling of cellular damage produced by ionizing radiation and that both are involved in the activation of p53. The suboptimal p53-mediated response could contribute to the high cancer risk and radiosensitivity seen in NBS patients.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 05/12/20 alle ore 02:09:09