The Autoantibodies to α6β4 Integrin of Patients Affected by Ocular Cicatricial Pemphigoid Recognize Predominantly Epitopes Within the Large Cytoplasmic Domain of Human β4

摘要: This study was undertaken to characterize the antigenic determinants recognized by autoantibodies of patients with ocular cicatricial pemphigoid (OCP). OCP is a subepithelial, blistering, autoimmune disease that mainly affects conjunctiva and other mucous membranes. We previously demonstrated cDNA clone, isolated from keratinocyte expression library using immunoaffinity-purified autoantibody, encoded cytoplasmic domain β 4 integrin subunit. Our subsequent studies showed sera all were tested recognize human To identify prevalent epitopes anti-β OCP, we have used cell lines transfected vectors encoding wild-type subunit, tailless or subunit lacking extracellular domain. Nontransfected as controls. Lysates these analyzed sera, IgG fractions autoantibodies. Abs domains positive controls, whereas normal negative The reactivity determined immunoblotting, immunoprecipitation, FACS analysis. results this indicate fractions, react intracellular not In vitro culture experiments autoantibody binds cytoplasm cells. relevance findings pathogenesis discussed.