Antibodies to α5 chain of collagen IV are pathogenic in Goodpasture's disease
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- 作者：Zhao Cuia ; Ming-hui Zhaoa ; b ; mhzhao@bjmu.edu.cn" class="auth_mail" title="E-mail the corresponding author ; Xiao-yu Jiaa ; Miao Wanga ; Shui-yi Hua ; Su-xia Wanga ; Feng Yua ; Kyle L. Brownc ; d ; Billy G. Hudsonc ; d ; e ; f ; g ; h ; i ; billy.hudson@vanderbilt.edu" class="auth_mail" title="E-mail the corresponding author ; Vadim Pedchenkoc ; d
- 关键词：Goodpasture's disease ; Glomerular basement membrane (GBM) ; Autoantibody ; Collagen IV ; NC1 domain ; Antigen ; Epitopes
- 刊名：Journal of Autoimmunity
- 出版年：2016
- 出版时间：June 2016
- 年：2016
- 卷：70
- 期：Complete
- 页码：1-11
- 全文大小：2793 K
文摘
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Antibody against α5 non-collagenous domain (NC1) of collagen IV induces Goodpasture's (GP) disease.
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Autoantibodies target two conformational epitopes, EA and EB, in α5NC1 domain.
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Structural analysis identified key epitope residues for antibody binding.
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Immunization with α5NC1 domain induces glomerulonephritis and lung hemorrhage in WKY rats.
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Occurrence of autoantibodies targeting α3NC1 and α5NC1 presents a conundrum for the etiology of GP disease.