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肾小球基底膜肾病的病理学

Pathology of glomerular basement membrane nephropathy.

作者信息

Hudson B G, Kalluri R, Tryggvason K

机构信息

Department of Biochemistry and Molecular Biology, University of Kansas Medical Center, Kansas City 66160.

出版信息

Curr Opin Nephrol Hypertens. 1994 May;3(3):334-9. doi: 10.1097/00041552-199405000-00016.

Abstract

Studies of the molecular pathology of glomerular basement membrane have led to the discovery of four novel chains of type IV collagen, the latest of which is the alpha 6(IV) chain. Three chains have been shown to be directly involved in the pathogenesis of Goodpasture syndrome, Alport syndrome, and Alport posttransplantation antiglomerular basement membrane nephritis. This review focuses on the novel chains with respect to recent advances in their structure, gene organization, and involvement in the pathogenesis of these glomerulopathies.

摘要

对肾小球基底膜分子病理学的研究已导致发现了四种新的IV型胶原链,其中最新的是α6(IV)链。已表明有三种链直接参与了Goodpasture综合征、Alport综合征和Alport移植后抗肾小球基底膜肾炎的发病机制。本综述重点关注这些新链在结构、基因组织以及参与这些肾小球病发病机制方面的最新进展。

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