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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.

Abstract
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.
AuthorsD J Matthews, P A Clark, J Herbert, G Morgan, R J Armitage, C Kinnon, A Minty, K H Grabstein, D Caput, P Ferrara
JournalBlood (Blood) Vol. 85 Issue 1 Pg. 38-42 (Jan 01 1995) ISSN: 0006-4971 [Print] United States
PMID7803808 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Interleukin-13
  • Interleukin-15
  • Interleukin-2
  • Interleukins
  • Receptors, Interleukin-2
  • Interleukin-4
  • Immunoglobulin E
Topics
  • B-Lymphocytes (immunology)
  • Cell Division
  • Genetic Linkage
  • Humans
  • Immunoglobulin E (metabolism)
  • Infant
  • Interleukin-13 (pharmacology)
  • Interleukin-15
  • Interleukin-2 (pharmacology)
  • Interleukin-4 (pharmacology)
  • Interleukins (pharmacology)
  • Lymphocyte Activation
  • Male
  • Mutation
  • Polymerase Chain Reaction
  • Receptors, Interleukin-2 (chemistry, physiology)
  • Severe Combined Immunodeficiency (genetics, immunology)
  • Structure-Activity Relationship
  • X Chromosome

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