Wasp in immune-system organization and function

Abstract

Wiskott–Aldrich syndrome (WAS) is a rare X-linked disease that is characterized by immune dysregulation and micro-thrombocytopaenia. WAS protein (WASp) is a haematopoietic-restricted member of a family of proteins that transduce signals from the cell surface to the actin cytoskeleton. When activated by the Rho GTPase Cdc42, WASp binds to the cytoskeletal-organizing complex Arp2/3, which initiates polymerization of actin and formation of a branching network of filaments. WASp has many effects on the immune system and participates in antigen-receptor signalling, phagocytosis and cell migration. Many of the defects of WAS might be attributable to abnormal cell transport and cell–cell interactions.