The Role of the Rhomboid Superfamily in ER Protein Quality Control: From Mechanisms and Functions to Diseases
The Role of the Rhomboid Superfamily in ER Protein Quality Control: From Mechanisms and Functions to Diseases
复制标题
Rhomboid超家族在ER蛋白质量控制中的作用： 从机制和功能到疾病
DOI：
10.1101/cshperspect.a041248
复制
发表时间：
2023-02-01
影响因子：
7.2
通讯作者：
Neal, Sonya E. E.
中科院分区：
文献类型：
--
作者：
Bhaduri, Satarupa;Scott, Nicola A. A.;Neal, Sonya E. E.
内质网（ER）是真核细胞中一个重要的细胞器，是蛋白质折叠、修饰和脂质合成的主要场所。ER内的扰动，如蛋白质错误折叠和对蛋白质折叠的高需求，导致ER蛋白质质量控制网络的失调和ER应激。最近，菱形超家族已成为ER蛋白质质量控制的关键参与者，因为它具有多种细胞功能，包括ER相关降解（ERAD），内体高尔基体相关降解（EGAD）和ER抢先质量控制（ERpQC）。这种广泛的功能既说明了菱形超家族在健康和疾病中的重要性，也强调了了解其作用机制的必要性。由于菱形蛋白的调节异常与各种疾病如神经系统疾病和癌症有关，因此它们代表了有希望的潜在治疗药物靶点。本文综述了菱形蛋白在ER蛋白质量控制中的各种作用，并讨论了它们在健康和疾病中的意义。
The endoplasmic reticulum (ER) is an essential organelle in eukaryotic cells and is a major site for protein folding, modification, and lipid synthesis. Perturbations within the ER, such as protein misfolding and high demand for protein folding, lead to dysregulation of the ER protein quality control network and ER stress. Recently, the rhomboid superfamily has emerged as a critical player in ER protein quality control because it has diverse cellular functions, including ER-associated degradation (ERAD), endosome Golgi-associated degradation (EGAD), and ER preemptive quality control (ERpQC). This breadth of function both illustrates the importance of the rhomboid superfamily in health and diseases and emphasizes the necessity of understanding their mechanisms of action. Because dysregulation of rhomboid proteins has been implicated in various diseases, such as neurological disorders and cancers, they represent promising potential therapeutic drug targets. This review provides a comprehensive account of the various roles of rhomboid proteins in the context of ER protein quality control and discusses their significance in health and disease.