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Functional role of interleukin-4 (IL-4) and IL-7 in the development of X-linked severe combined immunodeficiency.

Authors
  • Kumaki, S
  • Ishii, N
  • Minegishi, M
  • Tsuchiya, S
  • Cosman, D
  • Sugamura, K
  • Konno, T
Type
Published Article
Journal
Blood
Publication Date
Jan 15, 1999
Volume
93
Issue
2
Pages
607–612
Identifiers
PMID: 9885222
Source
Medline
License
Unknown

Abstract

X-linked severe combined immunodeficiency (X-SCID) is characterized by an absent or diminished number of T cells and natural-killer (NK) cells with a normal or elevated number of B cells, and results from mutations of the gammac chain. The gammac chain is shared by interleukin-2 (IL-2), IL-4, IL-7, IL-9, and IL-15 receptors. Recently, a survival signal through the IL-7 receptor alpha (IL-7Ralpha) chain was shown to be important for T-cell development in mice and was suggested to contribute to the X-SCID phenotype. In the present study, we examined function of a mutant gammac chain (A156V) isolated from an X-SCID patient and found that T cells expressing the mutant gammac chain were selectively impaired in their responses to IL-4 or IL-7 compared with the wild-type gammac chain expressing cells although responses to IL-2 or IL-15 were relatively maintained. The result shows that IL-4- and/or IL-7-induced signaling through the gammac chain is critical for T-cell development and plays an important role in the development of the X-SCID phenotype.

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