CXCR4 signaling in health and disease

• The CXCR4 ligand: the role of CXCL12.
• CXCR4 structure and expression.
• CXCR4 homo and hetero-oligomerization.
• Physiologic role of CXCR4.
• CXCR4 in disease: the WHIM syndrome.

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.