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Cytokine-Mediated Regulation of Human Lymphocyte Development and Function: Insights from Primary Immunodeficiencies

Abstract

Cytokine-mediated intracellular signaling pathways are fundamental for the development, activation, and differentiation of lymphocytes. These distinct processes underlie protection against infectious diseases after natural infection with pathogens or immunization, thereby providing the host with long-lived immunological memory. In contrast, aberrant cytokine signaling can also result in conditions of immune dysregulation, such as early-onset autoimmunity. Thus, balanced signals provided by distinct cytokines, and delivered to specific cell subsets, are critical for immune homeostasis. The essential roles of cytokines in human immunity have been elegantly and repeatedly revealed by the discovery of individuals with mutations in cytokine ligands, receptors, and downstream transcription factors that cause primary immunodeficiency or autoimmune conditions. In this article, we review how the discovery and characterization of such individuals has identified nonredundant, and often highly specialized, functions of specific cytokines and immune cell subsets in human lymphocyte biology, host defense against infections, and immune regulation.

Type Journal
ISBN 1550-6606 (Electronic) 0022-1767 (Linking)
Authors Tangye, S. G.; Pelham, S. J.; Deenick, E. K.; Ma, C. S.
Responsible Garvan Author Prof Stuart Tangye
Publisher Name JOURNAL OF IMMUNOLOGY
Published Date 2017-09-15 00:00:00
Published Volume 199
Published Issue 6
Published Pages 1949-1958
Status Published in-print
DOI 10.4049/jimmunol.1700842
URL link to publisher's version https://www.ncbi.nlm.nih.gov/pubmed/28874415