Coiled-coil domain-containing protein 88B (CCDC88B)

CCDC88B encodes a cytoskeletal scaffold protein with a coiled-coil and hook-related domain architecture. It is primarily expressed in hematopoietic organs and immune cells, including T lymphocytes and myeloid lineages, but not B lymphocytes. It regulates T cell maturation, activation, and cytokine production, and controls NK cell cytotoxicity as well as dendritic cell migration through cytoskeleton interactions. CCDC88B interacts physically and functionally with other signaling molecules—including ARHGEF2, RASAL3, HSPA5, and DOCK8—that modulate immune responses, vesicular trafficking, and apoptosis. The gene is situated at chromosome locus 11q13, associated with risk for several inflammatory and autoimmune disorders. Its precise biochemical mechanisms are largely linked to its role as a cytoskeletal scaffold, coupling organelle transport and immune cell activation. Despite its strong linkage to disease susceptibility, it is not currently targeted by approved drugs and presents a complex safety profile due to its essential functions in immune regulation.

Other names

BRLZHkRP3FLJ37970FLJ00354GIPIEGipieBrain leucine zipper domain-containing proteinHook-related protein 3GRP78-interacting protein induced by ER stressCCDC88HKRP378 kDa glucose-regulated protein [GRP78]-interacting protein induced by ER stressCoiled-coil domain containing 88