194 Phospholipase Cγ2 is Essential in Autoantibody-induced Skin Blistering

Abstract

Phospholipase Cγ2 (PLCγ2) is a signaling molecule of tyrosine kinase-coupled receptors in cells of hematopoietic origin. Gain-of-function mutations of PLCγ2 have been reported to cause a complex syndrome involving inflammation and bullous skin eruptions in both mice and humans. Previously we showed that PLCγ2 deficient mice remained protected in an autoimmune skin blistering model induced by the passive transfer of autoantibodies against collagen type VII (C7), which is a key anchoring fibril in the dermal-epidermal junction.