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Titolo:
MULTIPLE GENETIC-LOCI WITHIN 11P15 DEFINED BY BECKWITH-WIEDEMANN SYNDROME REARRANGEMENT BREAKPOINTS AND SUBCHROMOSOMAL TRANSFERABLE FRAGMENTS
Autore:
HOOVERS JMN; KALIKIN LM; JOHNSON LA; ALDERS M; REDEKER B; LAW DJ; BLIEK J; STEENMAN M; BENEDICT M; WIEGANT J; LENGAUER C; TAILLONMILLER P; SCHLESSINGER D; EDWARDS MC; ELLEDGE SJ; IVENS A; WESTERVELD A; LITTLE P; MANNENS M; FEINBERG AP;
Indirizzi:
JOHNS HOPKINS UNIV,SCH MED,DEPT MED ONCOL,720 RUTLAND AVE BALTIMORE MD 21205 JOHNS HOPKINS UNIV,SCH MED,DEPT MED ONCOL BALTIMORE MD 21205 JOHNS HOPKINS UNIV,SCH MED,DEPT MOLEC BIOL & GENET BALTIMORE MD 21205 UNIV AMSTERDAM,ACAD MED CTR,INST HUMAN GENET 1105 AZ AMSTERDAM NETHERLANDS UNIV MICHIGAN,MOLEC & CELLULAR BIOL PROGRAM ANN ARBOR MI 48109 LEIDEN UNIV,DEPT CYTOCHEM & CYTOMETRY LEIDEN NETHERLANDS UNIV HEIDELBERG,INST HUMAN GENET & ANTHROPOL W-6900 HEIDELBERG GERMANY WASHINGTON UNIV,DEPT MOLEC MICROBIOL ST LOUIS MO 63110 BAYLOR COLL MED,HOWARD HUGHES MED INST,DEPT BIOCHEM HOUSTON TX 77030 UNIV LONDON IMPERIAL COLL SCI & TECHNOL,DEPT BIOCHEM LONDON ENGLAND
Titolo Testata:
Proceedings of the National Academy of Sciences of the United Statesof America
fascicolo: 26, volume: 92, anno: 1995,
pagine: 12456 - 12460
SICI:
0027-8424(1995)92:26<12456:MGW1DB>2.0.ZU;2-N
Fonte:
ISI
Lingua:
ENG
Soggetto:
WILMS-TUMOR; CLONES; CHROMOSOME-11; RELAXATION;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
34
Recensione:
Indirizzi per estratti:
Citazione:
J.M.N. Hoovers et al., "MULTIPLE GENETIC-LOCI WITHIN 11P15 DEFINED BY BECKWITH-WIEDEMANN SYNDROME REARRANGEMENT BREAKPOINTS AND SUBCHROMOSOMAL TRANSFERABLE FRAGMENTS", Proceedings of the National Academy of Sciences of the United Statesof America, 92(26), 1995, pp. 12456-12460

Abstract

Beckwith-Wiedemann syndrome (BWS) involves fetal overgrowth and predisposition to a wide variety of embryonal tumors of childhood. We have previously found that BWS is genetically linked to 11p15 and that thissame band shows loss of heterozygosity in the types of tumors to which children with BWS are susceptible, However, 11p15 contains >20 megabases, and therefore, the BWS and tumor suppressor genes could be distinct, To determine the precise physical relationship between these loci, we isolated yeast artificial chromosomes, and cosmid libraries from them, within the region of loss of heterozygosity in embryonal tumors. Five germ-fine balanced chromosomal rearrangement breakpoint sites from BWS patients, as well as a balanced chromosomal translocation breakpoint from a rhabdoid tumor, were isolated within a 295- to 320-kb cluster defined by a complete cosmid contig crossing these breakpoints. This breakpoint cluster terminated approximately 100 kb centromeric to the imprinted gene IGF2 and 100 kb telomeric to p57(KIP2), an inhibitor of cyclin-dependent kinases, and was located within subchromosomal transferable fragments that suppressed the growth of embryonal tumor cells in genetic complementation experiments. We have identified 11 transcribed sequences in this BWS/tumor suppressor coincident region, one of which corresponded to p57(KIP2). However, three additional BWS breakpoints were >4 megabases centromeric to the other five breakpoints and were excluded from the tumor suppressor region defined by subchromosomal transferable fragments, Thus, multiple genetic loci define BWS and tumor suppression on 11p15.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 01/12/20 alle ore 01:16:50