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.
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Authors | D J Matthews, P A Clark, J Herbert, G Morgan, R J Armitage, C Kinnon, A Minty, K H Grabstein, D Caput, P Ferrara |
Journal | Blood
(Blood)
Vol. 85
Issue 1
Pg. 38-42
(Jan 01 1995)
ISSN: 0006-4971 [Print] United States |
PMID | 7803808
(Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Interleukin-13
- Interleukin-15
- Interleukin-2
- Interleukins
- Receptors, Interleukin-2
- Interleukin-4
- Immunoglobulin E
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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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