Characterization of Autoantibodies in Pemphigus Using Antigen-Specific Enzyme-Linked Immunosorbent Assays with Baculovirus-Expressed Recombinant Desmogleins

Ken Ishii, Masayuki Amagai, Russell P. Hall, Takashi Hashimoto, Atsushi Takayanagi, Shinobu Gamou, Nobuyoshi Shimizu, Takeji Nishikawa

研究成果: Article査読

437 被引用数 (Scopus)

抄録

Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune skin diseases caused by autoantibodies against desmoglein (Dsg) 3 and Dsg1, respectively. Routine immunofluorescence testing of skin and serum from patients cannot distinguish between these two severe diseases since both have IgG Abs directed against keratinocyte cell surfaces. In this study, recombinant Dsg3 and Dsg1, produced as secreted proteins by baculovirus expression, have been utilized to develop ELISAs for the specific characterization of their autoantibodies. Of 49 PV sera, 46 were positive in the Dsg3 ELlSA and 44 of 46 PF sera were positive in the Dsg1 ELISA, compared with only 3 of 23 sera of bullous pemphigoid, and none of 53 normal control sera in both ELISAs. Both the Dsg3 and Dsg1 ELISAs were more specific and sensitive than conventional immunofluorescence staining. These Ag-specific ELISAs revealed that more than one-half of PV sera (26 of 49) had anti-Dsg1 Abs in addition to anti-Dsg3 Abs. PV patients who had not only oral mucous lesions but also significant skin involvement tended to have higher titers of anti-Dsg1 Abs. Furthermore, the ELISA reactivity correlated well with clinical disease activity in 5 of 6 PV and 5 of 5 PF patients. This ELISA provides a sensitive and highly specific assay for the diagnosis of patients with PV and PF, the correlation of disease activity with serum Ab levels, and a novel tool for investigating the immunopathogenesis of pemphigus.

本文言語English
ページ(範囲)2010-2017
ページ数8
ジャーナルJournal of Immunology
159
4
出版ステータスPublished - 1997 8 15

ASJC Scopus subject areas

  • 免疫アレルギー学
  • 免疫学

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