Pemphigus Vulgaris Autoantibodies Induce an Endoplasmic Reticulum Stress Response.

ABSTRACT: Desmosomes are intercellular junctions that mediate cell-cell adhesion and are essential for maintaining tissue integrity. Pemphigus vulgaris (PV) is an autoimmune epidermal blistering disease caused by autoantibodies (IgG) targeting desmoglein 3, a desmosomal cadherin. PV autoantibodies cause desmosome disassembly and loss of cell-cell adhesion; however, the molecular signaling pathways that regulate these processes are not fully understood. Using high-resolution time-lapse imaging of live keratinocytes, we found that endoplasmic reticulum (ER) tubules make frequent and persistent contacts with internalizing desmoglein 3 puncta in keratinocytes treated with IgG of patients with PV. Biochemical experiments demonstrated that PV IgG activated ER stress signaling pathways, including both IRE1

SUBMITTER: Hoffman CL 

PROVIDER: S-EPMC12317713 | biostudies-literature | 2025 Sep

REPOSITORIES: biostudies-literature