Correlation of peptide specificity and IgG subclass with pathogenic and nonpathogenic autoantibodies in pemphigus vulgaris: a model for autoimmunity.

Pemphigus vulgaris Subclass Desmoglein 3 Acantholysis
DOI: 10.1073/pnas.92.11.5239 Publication Date: 2006-05-31T13:11:38Z
ABSTRACT
Pemphigus vulgaris (PV) is a rare, potentially fatal, autoimmune disease that affects the skin and mucous membranes. The PV antigen (PVA) has been characterized as desmoglein 3. patients carry HLA-DR4- or HLA-DR6-bearing extended haplotypes. We recently demonstrated with active have high titers of autoantibodies IgG1 IgG4 subclasses. Patients in remission, healthy unaffected relatives, some MHC-matched normal individuals low levels autoantibodies, which are only. Furthermore, intraperitoneal injection IgG from caused clinical mice, but did not. prepared 12 peptides 30 amino acids each (peptides Bos 1-12) spanning extracellular domain PVA. recognize 1 6 autoantibodies. remission to peptide only, statistically significantly lower (P < 0.01). They no longer subclass 6. Healthy relatives unrelated only 1. In vitro studies indicate 6-specific and, lesser extent, 1-specific can cause acantholysis. Our data suggest probably main acantholytic autoantibody, while may act facilitator enhancer process. this study we illustrate paradigms demonstrate interactions between MHC, specificities Thus, provides an important model pathogenesis autoimmunity.
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