首页|期刊导航|中华医学杂志（英文版）|Role of autoantibodies against the linker subdomains of envoplakin and periplakin in the pathogenesis of paraneoplastic pemphigus
中华医学杂志（英文版）2009，Vol.122Issue(5)：486-495,10.DOI:10.3760/cma.j.issn.0366-6999.2009.05.002
Role of autoantibodies against the linker subdomains of envoplakin and periplakin in the pathogenesis of paraneoplastic pemphigus
Role of autoantibodies against the linker subdomains of envoplakin and periplakin in the pathogenesis of paraneoplastic pemphigus
摘要
Abstract
Background The presence of autoantibodies against multiple epidermal proteins is an important feature in paraneoplastic pemphigus (PNP). Circulating anti-desmoglein 3 autoantibody, the major pathogenic autoantibody in pemphigus vulgaris (PV), has been proved pathogenic in PNP. Because of many clinical differences between PNP and PV, we speculate about the involvement of other autoantibodies in the pathogenesis of PNP. Envoplakin (EPL) and periplakin (PPL) are recognized by most PNP sere. Their linker subdomains are highly homologous and necessary for the association of intermediate filaments.Methods We characterized the autoantibodies against the linker subdomains of EPL and PPL in PNP patients' sera and their associated tumors by enzyme-linked immunosorbent assay (ELISA) and immunofluorence. We also applied the purified autoantibodies against EPL and PPL from PNP sera to cultured human epidermal keratinocytes (HEK), to evaluate the changes of cell-cell adhesion.Results Autoantibodies against EPL and PPL were detected in most PNP patients by ELISA, and the decrease of these autoantibodies after removal of the tumors was roughly comparable to the improvement of clinical symptoms. Cultured tumor cells from PNP patients secreted these autoantibodies. Specific immunoglobulin receptors for EPL and PPL were found on B lymphocytes in tumors from PNP. Furthermore, purified anti-EPL and anti-PPL autoantibodies from PNP sere were capable of dissociating cultured human epidermal keratinocytes.Conclusion Autoantibodies against EPL and PPL may also be pathogenic in PNP.关键词
envoplakin/periplakin/linker subdomain/paraneoplastic pemphigus/pathogenesisKey words
envoplakin/periplakin/linker subdomain/paraneoplastic pemphigus/pathogenesis分类
医药卫生引用本文复制引用
LI Jing,BU Ding-fang,HUANG Yong-chu,ZHU Xue-jun..Role of autoantibodies against the linker subdomains of envoplakin and periplakin in the pathogenesis of paraneoplastic pemphigus[J].中华医学杂志（英文版）,2009,122(5):486-495,10.基金项目
This work was supported by a grant from the National NaturalScience Foundation of China (No. 30671890). （No. 30671890）
