Chemokines and chemokine receptors regulate multiple processes such morphogenesis, angiogenesis and immune responses. Among the chemokine receptors, CXCR4 stands out for its pleiotropic roles as well as for its involvement in several pathological conditions, including immune diseases, viral infections and cancer. For these reasons, CXCR4 represents a crucial target in drug development. In this review, we discuss of CXCR4 receptor properties and signaling in health and diseases, focusing on the WHIM syndrome, an inherited immunodeficiency caused by mutations of the CXCR4 gene.
Keywords: CXCL12; CXCR4; Chemokine receptors; Immunity; Signaling pathway; WHIM syndrome.
Copyright © 2016 European Federation of Immunological Societies. Published by Elsevier B.V. All rights reserved.