ERp57，一种多功能内质网驻留氧化还原酶。

ERp57, a multifunctional endoplasmic reticulum resident oxidoreductase.

机构信息

Department of Biochemistry, University of Alberta, Edmonton, Alberta, Canada T6G 2H7.

出版信息

Int J Biochem Cell Biol. 2010 Jun;42(6):796-9. doi: 10.1016/j.biocel.2010.01.009. Epub 2010 Jan 15.

DOI:10.1016/j.biocel.2010.01.009

PMID:20079872

Abstract

ERp57 is a 58-kDa thiol oxidoreductase and a member of the protein disulfide isomerase (PDI)-like family. ERp57 is highly similar to other PDI family members in terms of amino acid sequence and structural/functional domain organization; however, it possesses some distinctive structural features that dictate its unique functions in the cell. This protein plays an important role in endoplasmic reticulum quality control of newly synthesized glycoproteins, is critical in major histocompatability complex (MHC) class I assembly and regulates gene expression. Studies on ERp57-deficient mice indicate that the protein is critical during embryonic development. The protein has been implicated in human pathologies including cancer and Alzheimer's disease.

摘要

ERp57 是一种 58kDa 的硫醇氧化还原酶，也是蛋白二硫键异构酶（PDI）样家族的成员。在氨基酸序列和结构/功能域组织方面，ERp57 与其他 PDI 家族成员非常相似；然而，它具有一些独特的结构特征，决定了其在细胞中的独特功能。这种蛋白质在新合成糖蛋白的内质网质量控制中发挥重要作用，在主要组织相容性复合体（MHC）I 类组装中至关重要，并调节基因表达。对 ERp57 缺陷型小鼠的研究表明，该蛋白在胚胎发育过程中至关重要。该蛋白已涉及包括癌症和阿尔茨海默病在内的人类病理学。