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Titolo:
WT1: what has the last decade told us?
Autore:
Little, M; Holmes, G; Walsh, P;
Indirizzi:
Univ Queensland, Ctr Cellular & Mol Biol, Brisbane, Qld 4072, Australia Univ Queensland Brisbane Qld Australia 4072 Brisbane, Qld 4072, Australia Univ Queensland, Dept Biochem, Brisbane, Qld 4072, Australia Univ Queensland Brisbane Qld Australia 4072 Brisbane, Qld 4072, Australia Univ Queensland, Ctr Drug Design & Dev, Brisbane, Qld, Australia Univ Queensland Brisbane Qld Australia n & Dev, Brisbane, Qld, Australia
Titolo Testata:
BIOESSAYS
fascicolo: 3, volume: 21, anno: 1999,
pagine: 191 - 202
SICI:
0265-9247(199903)21:3<191:WWHTLD>2.0.ZU;2-U
Fonte:
ISI
Lingua:
ENG
Soggetto:
WILMS-TUMOR GENE; DENYS-DRASH-SYNDROME; ACUTE MYELOID LEUKEMIAS; SUPPRESSOR GENE; DNA-BINDING; RNA-BINDING; PROTEIN WT1; TRANSCRIPTIONAL REPRESSION; SUBNUCLEAR LOCALIZATION; GROWTH SUPPRESSION;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
87
Recensione:
Indirizzi per estratti:
Indirizzo: Little, M Univ Queensland, Ctr Cellular & Mol Biol, Brisbane, Qld 4072, Australia Univ Queensland Brisbane Qld Australia 4072 Qld 4072, Australia
Citazione:
M. Little et al., "WT1: what has the last decade told us?", BIOESSAYS, 21(3), 1999, pp. 191-202

Abstract

When positionally cloned in late 1989, it was anticipated that mutations within the Wilms' tumour suppressor gene (WT1) would prove responsible for this common solid kidney cancer of childhood. Characterisation of the WT1 expression pattern and of the structure of the encoded protein isoforms and their mode of action has now spanned almost a decade. WT1 proteins act as nucleic acid-binding zinc finger-containing transcription factors involved inboth transactivation and repression. These activities are facilitated and constrained by interactions with other proteins. Expression analyses and knockout mice indicate that WT1 protein plays a critical role in normal kidney and gonad development. Specific constitutional WT1 mutations results in several urogenital anomaly syndromes. While only 10% of sporadic Wilms' tumours do display WT1 mutation, WT1 is mutated in other cancers, including acute myeloid leukaemia. Much is still to be determined in WT1 biology. The next decade will see at least three streams of attention. The first two, elucidation of the role of WT1 in RNA metabolism and the characterisation of further protein partners, may together explain the distinct tissue-specific functions of WT1. Finally, further research into the role of WT1 in haematopoiesis will improve our understanding of WT1 in leukaemia. BioEssays 21:191-202, 1999. (C) 1999 John Wiley & Sons, Inc.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 29/03/20 alle ore 15:25:21