Role of Src and Cortactin in Pemphigus Skin Blistering.

ABSTRACT: Autoantibodies against desmoglein (Dsg) 1 and Dsg3 primarily cause blister formation in the autoimmune disease pemphigus vulgaris (PV). Src was proposed to contribute to loss of keratinocyte cohesion. However, the role and underlying mechanisms are unclear and were studied here. In keratinocytes, cell cohesion in response to autoantibodies was reduced in Src-dependent manner by two patient-derived PV-IgG fractions as well as by AK23 but not by a third PV-IgG fraction, although Src was activated by all autoantibodies. Loss of cell cohesion was progredient in a timeframe of 24 h and AK23, similar to PV-IgG, interfered with reconstitution of cell cohesion after Ca²⁺-switch, indicating that the autoantibodies also interfered with desmosome assembly. Dsg3 co-localized along cell cont

SUBMITTER: Kugelmann D 

PROVIDER: S-EPMC6461052 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature