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学科主题: 临床医学
题名:
Role of autoantibodies against the linker subdomains of envoplakin and periplakin in the pathogenesis of paraneoplastic pemphigus
作者: Li Jing; Bu Ding-fang; Huang Yong-chu; Zhu Xue-jun
关键词: envoplakin ; periplakin ; linker subdomain ; paraneoplastic pemphigus ; pathogenesis
刊名: CHINESE MEDICAL JOURNAL
发表日期: 2009-03-05
DOI: 10.3760/cma.j.issn.0366-6999.2009.05.002
卷: 122, 期:5, 页:486-495
收录类别: SCI
文章类型: Article
WOS标题词: Science & Technology
类目[WOS]: Medicine, General & Internal
研究领域[WOS]: General & Internal Medicine
关键词[WOS]: DENDRITIC CELL-SARCOMA ; CULTURED HUMAN KERATINOCYTES ; NON-HODGKINS-LYMPHOMA ; PLAKIN FAMILY ; CORNIFIED ENVELOPE ; ANTIGEN COMPLEX ; PATIENT ; ANTIBODIES ; VULGARIS ; INTERNALIZATION
英文摘要:

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 PNR Because of many clinical differences between PNP and PV, we speculate about the involvement of other autoantibodies in the pathogenesis of PNR 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 inmost 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 PNR

语种: 英语
所属项目编号: 30671890
项目资助者: National Natural Science Foundation of China
WOS记录号: WOS:000263997400002
Citation statistics:
内容类型: 期刊论文
URI标识: http://ir.bjmu.edu.cn/handle/400002259/54824
Appears in Collections:北京大学第一临床医学院_皮肤性病科_期刊论文

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作者单位: Peking Univ, Hosp 1, Dept Dermatol, Beijing 100034, Peoples R China

Recommended Citation:
Li Jing,Bu Ding-fang,Huang Yong-chu,et al. Role of autoantibodies against the linker subdomains of envoplakin and periplakin in the pathogenesis of paraneoplastic pemphigus[J]. CHINESE MEDICAL JOURNAL,2009,122(5):486-495.
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