神经系统疾病中的泛素系统突变。

IF 11.6 1区生物学 Q1 BIOCHEMISTRY & MOLECULAR BIOLOGY

Trends in Biochemical Sciences Pub Date : 2024-10-01 DOI:10.1016/j.tibs.2024.06.011

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引用次数: 0

摘要

神经元泛素平衡影响着无数细胞蛋白质的命运，泛素平衡的破坏与各种神经系统疾病有关。泛素系统对神经元细胞的正常状态转换以及清除威胁细胞完整性的错误折叠或聚集蛋白至关重要。这篇文章回顾了我们对泛素系统（尤其是 E3 连接酶和去泛素化酶）成分在神经发育和神经退行性疾病中的破坏的认识现状和最新进展。具体重点是最近在酶表征方面取得进展的酶，包括确定酶-底物配对、干细胞和动物模型的使用，以及泛素相关疾病疗法的开发。

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Ubiquitin system mutations in neurological diseases

Neuronal ubiquitin balance impacts the fate of countless cellular proteins, and its disruption is associated with various neurological disorders. The ubiquitin system is critical for proper neuronal cell state transitions and the clearance of misfolded or aggregated proteins that threaten cellular integrity. This article reviews the state of and recent advancements in our understanding of the disruptions to components of the ubiquitin system, in particular E3 ligases and deubiquitylases, in neurodevelopmental and neurodegenerative diseases. Specific focus is on enzymes with recent progress in their characterization, including identifying enzyme-substrate pairs, the use of stem cell and animal models, and the development of therapeutics for ubiquitin-related diseases.

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来源期刊

Trends in Biochemical Sciences 生物-生化与分子生物学

CiteScore

22.90

自引率

0.70%

发文量

148

审稿时长

6-12 weeks

期刊介绍： For over 40 years, Trends in Biochemical Sciences (TIBS) has been a leading publication keeping readers informed about recent advances in all areas of biochemistry and molecular biology. Through monthly, peer-reviewed issues, TIBS covers a wide range of topics, from traditional subjects like protein structure and function to emerging areas in signaling and metabolism. Articles are curated by the Editor and authored by top researchers in their fields, with a focus on moving beyond simple literature summaries to providing novel insights and perspectives. Each issue primarily features concise and timely Reviews and Opinions, supplemented by shorter articles including Spotlights, Forums, and Technology of the Month, as well as impactful pieces like Science & Society and Scientific Life articles.