The human protein disulfide isomerase gene family.
The human protein disulfide isomerase gene family.
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人蛋白二硫化物异构酶基因家族。
DOI：
10.1186/1479-7364-6-6
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发表时间：
2012-07-05
期刊：
影响因子：
4.5
通讯作者：
Petersen DR
中科院分区：
文献类型：
--
作者：
Galligan JJ;Petersen DR
酶介导的二硫键形成是一个高度保守的过程，影响超过三分之一的真核蛋白质。主要负责促进硫醇-二硫交换的酶是一个不断扩大的蛋白质家族的成员，称为蛋白质二硫异构酶（pdi）。这些蛋白质是一个更大的蛋白质超家族的一部分，被称为硫氧还蛋白家族（TRX）。作为PDI蛋白家族的成员，所有蛋白都含有trx样结构域，主要在内质网中表达。然而，亚细胞定位和TRX结构域的存在构成了基因家族分类所需的简短区分特征列表。迄今为止，PDI基因家族包含21个成员，在结构域组成、分子量、组织表达和细胞加工方面各不相同。鉴于它们在蛋白质折叠中的重要作用，PDI活性的丧失与许多疾病状态的发病机制有关，最常见的与未折叠蛋白反应（UPR）有关。在过去的十年中，UPR已成为包括阿尔茨海默病、帕金森病、酒精性和非酒精性肝病以及2型糖尿病在内的多种疾病的非常有吸引力的治疗靶点。了解蛋白质折叠的机制，特别是硫醇-二硫交换，可能会导致开发一类新的治疗方法，通过靶向普遍定期审议，有助于减轻多种疾病。
Enzyme-mediated disulfide bond formation is a highly conserved process affecting over one-third of all eukaryotic proteins. The enzymes primarily responsible for facilitating thiol-disulfide exchange are members of an expanding family of proteins known as protein disulfide isomerases (PDIs). These proteins are part of a larger superfamily of proteins known as the thioredoxin protein family (TRX). As members of the PDI family of proteins, all proteins contain a TRX-like structural domain and are predominantly expressed in the endoplasmic reticulum. Subcellular localization and the presence of a TRX domain, however, comprise the short list of distinguishing features required for gene family classification. To date, the PDI gene family contains 21 members, varying in domain composition, molecular weight, tissue expression, and cellular processing. Given their vital role in protein-folding, loss of PDI activity has been associated with the pathogenesis of numerous disease states, most commonly related to the unfolded protein response (UPR). Over the past decade, UPR has become a very attractive therapeutic target for multiple pathologies including Alzheimer disease, Parkinson disease, alcoholic and non-alcoholic liver disease, and type-2 diabetes. Understanding the mechanisms of protein-folding, specifically thiol-disulfide exchange, may lead to development of a novel class of therapeutics that would help alleviate a wide range of diseases by targeting the UPR.
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内质网相关降解和新型内质网应激反应基因derlin-3的作用。
影响因子：
20.1
作者：
Belmont PJ;Chen WJ;San Pedro MN;Thuerauf DJ;Gellings Lowe N;Gude N;Hilton B;Wolkowicz R;Sussman MA;Glembotski CC
通讯作者：
Glembotski CC
影响因子：
4.8
作者：
Alanen, Heli I.;Williamson, Richard A.;Ruddock, Lloyd W.
通讯作者：
Ruddock, Lloyd W.
影响因子：
3.4
作者：
Györke, I;Hester, N;Györke, S
通讯作者：
Györke, S
影响因子：
4.8
作者：
Adam, PJ;Boyd, R;Terrett, JA
通讯作者：
Terrett, JA
DOI：
10.1152/ajpendo.00053.2009
发表时间：
2009-09-01
影响因子：
5.1
作者：
Alberti, Avra;Karamessinis, Panagiotis;Charonis, Aristidis
通讯作者：
Charonis, Aristidis