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解析肾小球足细胞裂孔隔膜的分子组成。

Unraveling the molecular make-up of the glomerular podocyte slit diaphragm.

作者信息

Khoshnoodi J, Tryggvason K

机构信息

Division of Matrix Biology, Department of Medical Biochemistry and Biophysics, Karolinska Institutet, Stockholm, Sweden.

出版信息

Exp Nephrol. 2001;9(6):355-9. doi: 10.1159/000052632.

Abstract

Recent discoveries in podocyte proteins involved in the renal filtration barrier have shed new light on the ultrastructure of the kidney filter and pathogenesis of proteinuria. The identification of nephrin, a component of the slit diaphragm, and the intracellular slit diaphragm associated proteins CD2AP and podocin has demonstrated the existence of proteins that directly contribute to a functional kidney filter. Mutations in the genes for these three proteins result in proteinuria and nephrotic syndrome, and these proteins are also likely to be involved more generally in the pathomechanisms of proteinuria. This new knowledge has been promoted particularly through the powerful methods of molecular genetics and molecular biology. In this minireview, we present the recent progress in research of the podocyte slit diaphragm.

摘要

近期在参与肾滤过屏障的足细胞蛋白方面的发现,为肾滤器的超微结构及蛋白尿的发病机制带来了新的认识。裂孔隔膜成分nephrin以及细胞内裂孔隔膜相关蛋白CD2AP和足突融合蛋白的鉴定,证实了直接促成功能性肾滤器的蛋白质的存在。这三种蛋白质的基因突变会导致蛋白尿和肾病综合征,并且这些蛋白质也可能更广泛地参与蛋白尿的发病机制。尤其是通过强大的分子遗传学和分子生物学方法,这一新知识得到了推动。在本综述中,我们介绍了足细胞裂孔隔膜研究的最新进展。

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