학술논문
Interleukin-7 receptorα (IL-7Rα) deficiency: cellular and molecular bases. Analysis of clinical, immunological, and molecular features in 16 novel patients.
Document Type
Article
Author
Source
Subject
*INTERLEUKINS
*GROWTH factors
*IMMUNOLOGY
*CYTOKINES
*IMMUNE system
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Language
ISSN
0105-2896
Abstract
Analysis of gene-targeted mice and patients with severe combined immunodeficiency due to mutations of theα chain of the interleukin-7 receptor (IL-7Rα) 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-7Rα 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-7Rα deficiency and discuss the results of hematopoietic stem cell transplantation. [ABSTRACT FROM AUTHOR]