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分离出参与肺出血肾炎综合征的特异性肾小球基底膜抗原。

Isolation of the specific glomerular basement membrane antigen involved in Goodpasture syndrome.

作者信息

Wieslander J, Bygren P, Heinegård D

出版信息

Proc Natl Acad Sci U S A. 1984 Mar;81(5):1544-8. doi: 10.1073/pnas.81.5.1544.

Abstract

The antigen involved in the glomerulonephritis associated with antibodies to glomerular basement membrane (GBM) was purified from human GBM digested with highly purified clostridial collagenase. The purified nonreduced sample contained two components with closely similar mobilities on sodium dodecyl sulfate/polyacrylamide gel electrophoresis. After reduction they moved as one, nonantigenic, component, corresponding to a molecular weight of 26,000. Immunologically identical aggregates of higher molecular weight (i.e., 48,000) were also identified in the crude digest. Reduction of such aggregates after purification released some protein with a molecular weight of 26,000, but a large proportion was insensitive to reduction. Seven patients with Goodpasture syndrome all had circulating anti-GBM antibodies directed only against the purified antigen.

摘要

从用高度纯化的梭状芽孢杆菌胶原酶消化的人肾小球基底膜(GBM)中纯化出与抗肾小球基底膜(GBM)抗体相关的肾小球肾炎所涉及的抗原。纯化的非还原样品在十二烷基硫酸钠/聚丙烯酰胺凝胶电泳上含有两个迁移率非常相似的成分。还原后,它们作为一个非抗原性成分移动,对应分子量为26,000。在粗消化物中也鉴定出免疫相同的高分子量(即48,000)聚集体。纯化后这种聚集体的还原释放出一些分子量为26,000的蛋白质,但很大一部分对还原不敏感。7例Goodpasture综合征患者均具有仅针对纯化抗原的循环抗GBM抗体。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/07b3/344873/69f420e2de84/pnas00606-0266-a.jpg

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