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Function of the interleukin-2 (IL-2) receptor gamma-chain in biologic
responses of X-linked severe combined immunodeficient B cells to IL-2,
IL-4, IL-13, and IL-15
DJ Matthews, PA Clark, J Herbert, G Morgan, RJ Armitage, C Kinnon, A Minty, KH Grabstein, D Caput and P Ferrara
Cellular Immunology Unit, Institute of Child Health, London, UK.
The interleukin-2 (IL-2) receptor gamma-chain is a common component of
several members of the cytokine receptor superfamily including those for
IL-2, IL-4, IL-7, IL-9, IL-15, and possibly IL-13, and has recently been
renamed the common gamma-chain (gamma c-chain). Transfection experiments
have shown that the gamma c-chain participates in signal transduction by
IL-2, IL-4 and IL-7, but a functional role for the gamma c-chain in
biological responses by normal T cells and B cells to these cytokines has
not been established. In this study, we have used X- linked severe combined
immunodeficiency (X-SCID) as a naturally occurring gamma c-chain gene
disruption model to examine the role of the gamma c-chain in human B-cell
responses to IL-2, IL-4, IL-13, and IL-15. Our experiments show that B
cells from two X-SCID patients with characterized gamma c-chain gene
mutations do not respond to IL-2 or IL- 15, but respond as well or better
than normal B cells to both IL-4 and IL-13 in assays for B-cell activation,
proliferation, and IgE secretion. This finding raises important questions
about the function of the gamma c-chain in receptors for IL-4 and IL-13,
and the nature of the immune defect in X-SCID.
Volume 85,
Issue 1,
pp. 38-42,
01/01/1995
Copyright © 1995 by The American Society of Hematology
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