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从肠道到肾脏:内因子在生理和疾病中的作用。

From bowel to kidneys: the role of cubilin in physiology and disease.

机构信息

Section of Cell Biology, Department of Biomedicine, Faculty of Health Sciences, Aarhus University, Aarhus, Denmark.

出版信息

Nephrol Dial Transplant. 2013 Feb;28(2):274-81. doi: 10.1093/ndt/gfs565. Epub 2013 Jan 4.

DOI:10.1093/ndt/gfs565
PMID:23291372
Abstract

Cubilin is a large endocytic receptor serving such diverse functions as the intestinal absorption of the intrinsic factor-B(12) complex and the renal proximal tubule reabsorption of filtered proteins including albumin, transferrin, vitamin D-binding protein and other important plasma carriers. Cubilin is a structurally unique, peripheral membrane protein, which depends on the membrane protein amnionless (AMN) for correct apical translocation. In addition, AMN appears important for efficient internalization of intrinsic factor-B(12) in the intestine, whereas in the proximal tubule cubilin interacts with another endocytic receptor, megalin, for effective reabsorption. The importance of cubilin has been demonstrated in several animal models of cubilin deficiency as well as in a variety of human diseases. Recent demonstration of cubilin in podocytes from various species awaits further clarification with respect to the functional role as well as its role in pathology.

摘要

巨球蛋白受体是一种大型内吞受体,具有多种功能,如肠道吸收内因子-B(12)复合物和肾脏近端小管对包括白蛋白、转铁蛋白、维生素 D 结合蛋白和其他重要血浆载体在内的滤过蛋白的重吸收。巨球蛋白受体是一种结构独特的外周膜蛋白,其正确的顶端转位依赖于膜蛋白无顶(AMN)。此外,AMN 似乎对肠道内内因子-B(12)的有效内化很重要,而在近端小管中,巨球蛋白受体与另一种内吞受体巨球蛋白相互作用,以实现有效的重吸收。在几种巨球蛋白缺乏症的动物模型以及多种人类疾病中都证明了巨球蛋白受体的重要性。最近在各种物种的足细胞中发现了巨球蛋白受体,这需要进一步阐明其功能作用及其在病理学中的作用。

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