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Titolo:
Translocations of the RAR alpha gene in acute promyelocytic leukemia
Autore:
Zelent, A; Guidez, F; Melnick, A; Waxman, S; Licht, JD;
Indirizzi:
Inst Canc Res, Leukaemia Res Fund Ctr, Chester Beatty Labs, London SW3 6JB, England Inst Canc Res London England SW3 6JB eatty Labs, London SW3 6JB, England Mt Sinai Med Ctr, Dept Med, Derald H Ruttenberg Canc Ctr, New York, NY 10029 USA Mt Sinai Med Ctr New York NY USA 10029 g Canc Ctr, New York, NY 10029 USA Mt Sinai Med Ctr, Dept Med, Div Med Oncol, New York, NY 10029 USA Mt SinaiMed Ctr New York NY USA 10029 Med Oncol, New York, NY 10029 USA
Titolo Testata:
ONCOGENE
fascicolo: 49, volume: 20, anno: 2001,
pagine: 7186 - 7203
SICI:
0950-9232(20011029)20:49<7186:TOTRAG>2.0.ZU;2-5
Fonte:
ISI
Lingua:
ENG
Soggetto:
ACID RECEPTOR-ALPHA; TRANS-RETINOIC ACID; MITOTIC APPARATUS PROTEIN; POLYMERASE CHAIN-REACTION; ZINC-FINGER PROTEIN; COLONY-STIMULATING FACTOR; MINIMAL RESIDUAL DISEASE; PML GROWTH-SUPPRESSOR; NON-HODGKINS-LYMPHOMA; FUSION MESSENGER-RNA;
Keywords:
PLZF; NuMA; NPM; STAT; differentiation; retinoic acid;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
211
Recensione:
Indirizzi per estratti:
Indirizzo: Zelent, A Inst Canc Res, Leukaemia Res Fund Ctr, Chester Beatty Labs, 237 Fulham Rd,London SW3 6JB, England Inst Canc Res 237 Fulham Rd London England SW3 6JB 6JB, England
Citazione:
A. Zelent et al., "Translocations of the RAR alpha gene in acute promyelocytic leukemia", ONCOGENE, 20(49), 2001, pp. 7186-7203

Abstract

Acute promyelocytic leukemia (APL) has been recognized as a distinct clinical entity for over 40 years. Although relatively rare among hematopoietic malignancies (approximately 10% of AML cases), this disease has attracted aparticularly good share of attention by becoming the first human cancer inwhich all-trans-retinoic acid (ATRA), a physiologically active derivative of vitamin A, was able to induce complete remission (CR). ATRA induced remission is not associated with rapid cell death, as in the case of conventional chemotherapy, but with a restoration of the 'normal' granulocytic differentiation pathway. With this remarkable medical success story APL has overnight become a paradigm for the differentiation therapy of cancer. A few years later, excitement with APL was further enhanced by the discovery that a cytogenetic marker for this disease, the t(15:17) reciprocal chromosomal translocation, involves a fusion between the retinoic acid receptor alpha (RAR alpha) gene and a previously unknown locus named promyelocytic leukemia (PML). Consequence of this gene rearrangement is expression of the PML-RAR alpha chimeric oncoprotein, which is responsible for the cellular transformation as well as ATRA response that is observed in APL. Since this initial discovery, a number of different translocation partner genes of RAR alpha have been reported in rarer cases of APL, strongly suggesting that disruptionof RAR alpha underlies its pathogenesis. This article reviews various rearrangements of the RAR alpha gene that have so far been described in literature, functions of the proteins encoded by the different RAR alpha partner loci, and implications that these may have for the molecular pathogenesis ofAPL.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 22/09/20 alle ore 09:56:17