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Titolo:
Interleukin-7 receptor alpha chain-dependent signaling is required for T-cell development - Basis for T-B+NK+ severe combined immunodeficiencies in humans
Autore:
Puel, A; Leonard, WJ;
Indirizzi:
NHLBI, Mol Immunol Lab, NIH, Bethesda, MD 20892 USA NHLBI Bethesda MD USA20892 Mol Immunol Lab, NIH, Bethesda, MD 20892 USA
Titolo Testata:
IMMUNOLOGY AND ALLERGY CLINICS OF NORTH AMERICA
fascicolo: 1, volume: 20, anno: 2000,
pagine: 51 -
SICI:
0889-8561(200002)20:1<51:IRACSI>2.0.ZU;2-U
Fonte:
ISI
Lingua:
ENG
Soggetto:
COMMON GAMMA-CHAIN; NATURAL-KILLER-CELLS; DEFECTIVE LYMPHOID DEVELOPMENT; JAK-3 JANUS KINASE; MICE LACKING JAK3; IL-2 RECEPTOR; DEFICIENT MICE; MURINE INTERLEUKIN-7; GROWTH-FACTOR; BETA-CHAIN;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
99
Recensione:
Indirizzi per estratti:
Indirizzo: Leonard, WJ NHLBI, Mol Immunol Lab, NIH, Bldg 10,Room 7N252,900 Rockville Pike, Bethesda, MD 20892 USA NHLBI Bldg 10,Room 7N252,900 Rockville Pike Bethesda MD USA 20892
Citazione:
A. Puel e W.J. Leonard, "Interleukin-7 receptor alpha chain-dependent signaling is required for T-cell development - Basis for T-B+NK+ severe combined immunodeficiencies in humans", IMMUNOL ALL, 20(1), 2000, pp. 51

Abstract

X-linked SCID (XSCID) is characterized by profoundly diminished T and natural killer (NK) cells. XSCID is caused by mutations in the common cytokine receptor gamma chain, gamma(c), which is a component of the receptors for IL-2, IL-4, IL-7, IL-9, and IL-15. A similar clinical and immunologic spectrum results from mutations in JAK3, which associates with gamma(c). This article reviews these findings, and summarizes more recent evidence that mutations in IL-7R alpha cause a form of SCID in which T-tell numbers diminishedprofoundly but NK cells were normal. These findings not only provide the first elucidation of a basis for T-B+NK+ SCID but also suggest that defective IL-7R alpha-dependent signaling accounts for the T-cell defect in XSCID and JAK3 deficiency. Other implications of these findings also are discussed.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 04/04/20 alle ore 21:48:53