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Titolo:
Molecular mechanisms underlying human synovial sarcoma development
Autore:
dos Santos, NR; de Bruijn, DRH; van Kessel, AG;
Indirizzi:
Univ Nijmegen Hosp, Dept Human Genet, NL-6500 HB Nijmegen, Netherlands Univ Nijmegen Hosp Nijmegen Netherlands NL-6500 HB Nijmegen, Netherlands
Titolo Testata:
GENES CHROMOSOMES & CANCER
fascicolo: 1, volume: 30, anno: 2001,
pagine: 1 - 14
SICI:
1045-2257(200101)30:1<1:MMUHSS>2.0.ZU;2-B
Fonte:
ISI
Lingua:
ENG
Soggetto:
POLYMERASE-CHAIN-REACTION; IN-SITU HYBRIDIZATION; PARAFFIN-EMBEDDED TISSUE; KRUPPEL-ASSOCIATED BOX; FLUORESCENCE INSITU HYBRIDIZATION; COMPARATIVE GENOMIC HYBRIDIZATION; SSX FUSION TRANSCRIPTS; POLYCOMB GROUP COMPLEX; SYT-SSX; SOFT-TISSUE;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
110
Recensione:
Indirizzi per estratti:
Indirizzo: van Kessel, AG Univ Nijmegen Hosp, Dept Human Genet, Geert Grooteplein 10,POB 9101, NL-6500 HB Nijmegen, Netherlands Univ Nijmegen Hosp Geert Grooteplein 10,POB 9101 Nijmegen Netherlands NL-6500 HB
Citazione:
N.R. dos Santos et al., "Molecular mechanisms underlying human synovial sarcoma development", GENE CHROM, 30(1), 2001, pp. 1-14

Abstract

Synovial sarcomas are rather common among soft-tissue tumors, occurring atany age but affecting mainly young adults. The vast majority of synovial sarcomas carries a t(X; 18)(p 11.2;q11.2) chromosomal translocation, in about one-third of the cases as the sole cytogenetic anomaly. Several studies have indicated that the t(X;18) translocation arises exclusively in synovialsarcomas, therefore being an excellent tool to diagnose this malignancy. The breakpoint-associated genes were recently isolated: SYT, from chromosome18, and SSX1 and SSX2, both from the X chromosome. This discovery enabled the detection of SYT-SSX fusion transcripts by specific reverse transcriptase-polymerase chain reactions. This molecular genetics methodology has now been applied to numerous tumor samples and has led to the finding that, in contrast to tumors carrying SYT-SSX2 fusions, SYT-SSX1-positive tumors moreoften exhibit a biphasic histology, show a higher proliferation rate, and are associated with a poorer clinical outcome. It has also been shown that the SYT and SSX proteins are localized in the nucleus, where they appear toplay a role in transcriptional regulation, SYT as an activator of transcription and the SSX proteins SYT interacts and colocalizes in the nucleus with the BRM protein, a transcriptional coactivator, and that the SSX proteinscolocalize in the nucleus with polycomb group proteins, which are transcriptional corepressors. Together, these studies have provided mechanistic clues about how the SYT-SSX fusion proteins may trigger synovial sarcoma development. (C) 2001 Wiley-Liss, Inc.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 05/04/20 alle ore 09:38:18