Abstract
Analysis of gene-targeted mice and patients with severe combined immunodeficiency due to mutations of the alpha chain of the interleukin-7 receptor (IL-7Ralpha) has shown important differences between mice and humans in the role played by IL-7 in lymphoid development. More recently, it has been shown that IL-7Ralpha is also shared by the receptor for another cytokine, thymic stromal lymphopoietin (TSLP). In this review, we discuss recent advances in IL-7- and TSLP-mediated signaling. We also report on the clinical and immunological features of 16 novel patients with IL-7Ralpha deficiency and discuss the results of hematopoietic stem cell transplantation.
Publication types
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Research Support, Non-U.S. Gov't
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Review
MeSH terms
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Amino Acid Sequence
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Animals
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B-Lymphocytes / immunology
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Cytokines / genetics
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Cytokines / immunology
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Female
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Humans
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Male
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Mice
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Molecular Sequence Data
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Mutation / genetics
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Receptors, Interleukin-7 / chemistry
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Receptors, Interleukin-7 / deficiency*
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Receptors, Interleukin-7 / genetics
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Severe Combined Immunodeficiency / diagnosis
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Severe Combined Immunodeficiency / genetics*
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Severe Combined Immunodeficiency / immunology*
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Signal Transduction
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T-Lymphocytes / immunology
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Thymic Stromal Lymphopoietin
Substances
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Cytokines
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Receptors, Interleukin-7
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interleukin-7 receptor, alpha chain
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Thymic Stromal Lymphopoietin