作者：丁超伟，郭畅，袁雅冬

单位：

050000河北省石家庄市，河北医科大学第二医院呼吸与危重症医 学二科 通信作者：袁雅冬，E-mail：yuanyd1108@163.com

单位（英文）：

Department of Respiratory and Critical Care Medicine, the Second Hospital of Hebei Medical University, Shijiazhuang 050000, China Corresponding author: YUAN Yadong, E-mail: yuanyd1108@163.com

关键词：

肺动脉高压; 泛素-蛋白酶体系统; 泛素蛋白连接酶类; 泛素连接酶E3; 去泛素化酶; 综述;

关键词（英文）：

Pulmonary hypertension; Ubiquitination-proteasome system; Ubiquitin-protein ligases; Ubiquitin ligase E3; Deubiquitinating enzyme; Review

中图分类号：

DOI：

10.12114/j.issn.1008-5971.2022.00.247

基金项目：

河北省重点研发计划项目（21377701D）

摘要：

泛素化是真核细胞中最重要的蛋白质翻译后修饰形式之一，可参与调节细胞的各种生理过程，包括细胞周期进程、细胞分化、细胞凋亡以及先天性和适应性免疫反应。目前越来越多的靶向泛素-蛋白酶体系统(UPS)的药物被开发出来。有证据表明，肺动脉高压(PH)发病过程中存在UPS功能紊乱，但其机制尚不明确，也无相关药物应用于临床。本文回顾了UPS的结构和功能，并对PH中功能异常的UPS相关蛋白及其作用机制进行综述，以为临床精准诊断及靶向治疗方案的制定提供新思路。

英文摘要：

【Abstract】 Ubiquitination, one of the most important types of post-translational protein modifications in eukaryotic cells, has played a role in regulating various physiological processes including cell cycle progression, cell differentiation, cell apoptosis, and innate and adaptive immune responses. At present, more and more drugs targeting ubiquitin proteasome system (UPS) have been developed. There is evidence that UPS dysfunction exists in the pathogenesis of pathogenesis of pulmonary hypertension (PH) , but its mechanisms was still unclear, and no related-drugs were applied in clinic. This paper reviews the the structure and function of UPS, and summarizes the functional abnormalities of UPS related protein in PH and their mechanism of action, so as to provide new ideas for accurate clinical diagnosis and the formulation of targeted therapy.

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