TY - JOUR
T1 - The interleukin-2 receptor γ chain
T2 - Its role in the multiple cytokine receptor complexes and T cell development in XSCID
AU - Sugamura, Kazuo
AU - Asao, Hironobu
AU - Kondo, Motonari
AU - Tanaka, Nobuyuki
AU - Ishii, Naoto
AU - Ohbo, Kazuyuki
AU - Nakamura, Masataka
AU - Takeshita, Toshikazu
PY - 1996
Y1 - 1996
N2 - Interleukin 2 (IL-2), a T cell-derived cytokine, targets a variety of cells to induce their growth, differentiation, and functional activation. IL- 2 inserts signals into the cells through IL-2 receptors expressed on cell surfaces to induce such actions. In humans, the functional IL-2 receptor consists of the subunit complexes of the α, β, and γ chains, or the β and γ chains. The third component, the γ chain, of IL-2 receptor plays a pivotal role in formation of the full-fledged IL-2 receptor; together with the β chain, the γ chain participates in increasing the IL-2 binding affinity and intracellular signal transduction. Moreover, the cytokine receptors for at least IL-2, IL-4, IL-7, IL-9, and IL-15 utilize the same γ chain as an essential subunit. Interestingly, mutations of the γ chain gene cause human X-linked severe combined immunodeficiency (XSCID) characterized by a complete or profound T cell defect. Among the cytokines sharing the γ chain, at least IL-7 is essentially involved in early T cell development in the mouse organ culture system. The molecular identification of the γ chain brought a grasp of the structures and functions of the cytokine receptor and an in-depth understanding of the cause of human XSCID. To investigate the mechanism of XSCID and development of gene therapy for XSCID, knockout mice for the γ chain gene were produced that showed similar but not exactly the same phenotypes as human XSCID.
AB - Interleukin 2 (IL-2), a T cell-derived cytokine, targets a variety of cells to induce their growth, differentiation, and functional activation. IL- 2 inserts signals into the cells through IL-2 receptors expressed on cell surfaces to induce such actions. In humans, the functional IL-2 receptor consists of the subunit complexes of the α, β, and γ chains, or the β and γ chains. The third component, the γ chain, of IL-2 receptor plays a pivotal role in formation of the full-fledged IL-2 receptor; together with the β chain, the γ chain participates in increasing the IL-2 binding affinity and intracellular signal transduction. Moreover, the cytokine receptors for at least IL-2, IL-4, IL-7, IL-9, and IL-15 utilize the same γ chain as an essential subunit. Interestingly, mutations of the γ chain gene cause human X-linked severe combined immunodeficiency (XSCID) characterized by a complete or profound T cell defect. Among the cytokines sharing the γ chain, at least IL-7 is essentially involved in early T cell development in the mouse organ culture system. The molecular identification of the γ chain brought a grasp of the structures and functions of the cytokine receptor and an in-depth understanding of the cause of human XSCID. To investigate the mechanism of XSCID and development of gene therapy for XSCID, knockout mice for the γ chain gene were produced that showed similar but not exactly the same phenotypes as human XSCID.
KW - T cell development
KW - XSCID
KW - cytokine receptor
KW - interleukin 2
KW - signal transduction
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U2 - 10.1146/annurev.immunol.14.1.179
DO - 10.1146/annurev.immunol.14.1.179
M3 - Review article
C2 - 8717512
AN - SCOPUS:0029962080
VL - 14
SP - 179
EP - 205
JO - Annual Review of Immunology
JF - Annual Review of Immunology
SN - 0732-0582
ER -