Integrin alpha 6 beta 4

Integrin alpha 6 beta 4 is the core laminin receptor of hemidesmosomes, anchoring basal keratinocytes of skin, cornea and other stratified epithelia to the underlying basement membrane. The beta-4 subunit is unusual among integrins in possessing an exceptionally long cytoplasmic tail that connects to intermediate filaments through plectin and BP230 rather than to the actin cytoskeleton. Its principal ligand is laminin-332. Beyond mechanical adhesion, the receptor transduces survival and proliferative signals and is frequently amplified in carcinoma, where it promotes invasion and associates with poor prognosis. Mutations in ITGB4 cause epidermolysis bullosa with pyloric atresia, a severe inherited blistering disease of neonates.