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)
Chinese Medical Journal
;
(24): 486-495, 2009.
Artigo
em Inglês
| WPRIM
| ID: wpr-311835
ABSTRACT
<p><b>BACKGROUND</b>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.</p><p><b>METHODS</b>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.</p><p><b>RESULTS</b>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.</p><p><b>CONCLUSION</b>Autoantibodies against EPL and PPL may also be pathogenic in PNP.</p>
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Síndromes Paraneoplásicas
/
Farmacologia
/
Precursores de Proteínas
/
Autoanticorpos
/
Ensaio de Imunoadsorção Enzimática
/
Queratinócitos
/
Adesão Celular
/
Células Cultivadas
/
Pênfigo
/
Biologia Celular
Tipo de estudo:
Estudo de etiologia
Limite:
Adolescente
/
Adulto
/
Feminino
/
Humanos
/
Masculino
Idioma:
Inglês
Revista:
Chinese Medical Journal
Ano de publicação:
2009
Tipo de documento:
Artigo
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