Role of autoantibodies against the linker subdomains of envoplakin and periplakin in the pathogenesis of paraneoplastic pemphigus

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 clinic...

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Bibliographic Details
Published in:Chinese medical journal 2009-03, Vol.122 (5), p.486-495
Main Authors: Li, Jing, Bu, Ding-fang, Huang, Yong-chu, Zhu, Xue-jun
Format: Article
Language:English
Subjects:
Adolescent
Adult
Autoantibodies - immunology
Autoantibodies - pharmacology
Cell Adhesion - drug effects
Cells, Cultured
Desmoglein 3 - immunology
ELISA法
Enzyme-Linked Immunosorbent Assay
Epidermis - cytology
Female
Humans
Keratinocytes - cytology
Keratinocytes - drug effects
Male
Membrane Proteins - immunology
Middle Aged
Paraneoplastic Syndromes - immunology
Paraneoplastic Syndromes - metabolism
Pemphigus - immunology
Pemphigus - metabolism
Plakins - immunology
Protein Precursors - immunology
Young Adult
人表皮角质形成细胞
副肿瘤性天疱疮
发病机制
培养的肿瘤
自身抗体
酶联免疫吸附试验
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Summary: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 sera. 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 sera were capable of dissociating cultured human epidermal keratinocytes. Conclusion Autoantibodies against EPL and PPL may also be pathogenic in PNP.
ISSN:0366-6999
2542-5641
DOI:10.3760/cma.j.issn.0366-6999.2009.05.002