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破解遗传疾病的单泛素化密码

Cracking the Monoubiquitin Code of Genetic Diseases.

机构信息

VIB-KU Leuven Center for Cancer Biology, VIB, Herestraat 49, 3000 Leuven, Belgium.

Department of Oncology, KU Leuven, Herestraat 49, 3000 Leuven, Belgium.

出版信息

Int J Mol Sci. 2020 Apr 25;21(9):3036. doi: 10.3390/ijms21093036.

DOI:10.3390/ijms21093036
PMID:32344852
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7246618/
Abstract

Ubiquitination is a versatile and dynamic post-translational modification in which single ubiquitin molecules or polyubiquitin chains are attached to target proteins, giving rise to mono- or poly-ubiquitination, respectively. The majority of research in the ubiquitin field focused on degradative polyubiquitination, whereas more recent studies uncovered the role of single ubiquitin modification in important physiological processes. Monoubiquitination can modulate the stability, subcellular localization, binding properties, and activity of the target proteins. Understanding the function of monoubiquitination in normal physiology and pathology has important therapeutic implications, as alterations in the monoubiquitin pathway are found in a broad range of genetic diseases. This review highlights a link between monoubiquitin signaling and the pathogenesis of genetic disorders.

摘要

泛素化是一种普遍存在且动态的翻译后修饰,其中单个泛素分子或多泛素链被连接到靶蛋白上,分别导致单泛素化或多泛素化。在泛素领域的大多数研究都集中在降解性多泛素化上,而最近的研究揭示了单泛素修饰在重要生理过程中的作用。单泛素化可以调节靶蛋白的稳定性、亚细胞定位、结合特性和活性。了解单泛素化在正常生理和病理中的功能具有重要的治疗意义,因为在广泛的遗传疾病中发现了单泛素途径的改变。本综述强调了单泛素信号与遗传疾病发病机制之间的联系。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1a7/7246618/e2016431f39d/ijms-21-03036-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1a7/7246618/e2016431f39d/ijms-21-03036-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1a7/7246618/e2016431f39d/ijms-21-03036-g001.jpg

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