Abstract：
Retinal degeneration (RD) is a group of chronic blinding diseases characterised by progressive retinal cell death. As the disease progresses, vision deteriorates due to retinal cell death and impaired retinal integrity, eventually leading to complete loss of vision. Therefore, the function and environmental homeostasis of the retina have an important impact on the pathogenesis and treatment of RD. Ubiquitination, as a complex post-translational modification process, plays an essential role in maintaining retinal homeostasis and normal function. It covalently combines ubiquitin with protein through a series of enzyme-mediated reactions, and participates in cell processes such as gene transcription, cell cycle process, DNA repair, apoptosis and immune response. At the same time, it plays a central role in protein degradation. There are two major protein degradation systems in eukaryotic cells: the ubiquitin-proteasome system and the autophagy-lysosomal system. The protein degradation pathway maintains retinal protein homeostasis by reducing abnormal protein accumulation in the retina through two modes of degradation. Either dysregulation of ubiquitination or disruption of protein homeostasis may lead to the development of RD. This article aims to comprehensively review recent research progress on ubiquitin-related genes, proteins and protein homeostasis in the pathogenesis of RD, and to summarize the potential targeted therapy strategies for it. The review is expected to provide valuable guidance for further development and application of ubiquitination in RD.
摘要：
视网膜变性(RD)是一组以进行性视网膜细胞死亡为特征的慢性致盲疾病。随着疾病的进展,由于视网膜细胞死亡和受损的视网膜完整性，视力恶化，最终导致视力完全丧失。因此,视网膜的功能和环境稳态对RD的发病机制和治疗有重要影响。泛素化,作为一个复杂的翻译后修饰过程，在维持视网膜稳态和正常功能中起着至关重要的作用。它通过一系列酶介导的反应将泛素与蛋白质共价结合，并参与细胞过程，如基因转录，细胞周期过程，DNA修复,细胞凋亡和免疫反应。同时,它在蛋白质降解中起着核心作用。真核细胞中有两种主要的蛋白质降解系统：泛素-蛋白酶体系统和自噬-溶酶体系统。蛋白质降解途径通过两种降解模式减少视网膜中的异常蛋白质积累来维持视网膜蛋白质稳态。泛素化的失调或蛋白质稳态的破坏都可能导致RD的发展。本文旨在全面综述泛素相关基因的研究进展,RD发病机制中的蛋白质和蛋白质稳态，并总结其潜在的靶向治疗策略。该综述有望为泛素化在RD中的进一步发展和应用提供有价值的指导。
