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DOI10.1126/sciadv.adk3931
RNF220-mediated K63-linked polyubiquitination stabilizes Olig proteins during oligodendroglial development and myelination
Li, Yuwei; Wan, Li Pear; Song, Ning-Ning; Ding, Yu-Qiang; Zhao, Shuhua; Niu, Jianqin; Mao, Bingyu; Sheng, Nengyin; Ma, Pengcheng
发表日期2024
ISSN2375-2548
起始页码10
结束页码6
卷号10期号:6
英文摘要Maldevelopment of oligodendroglia underlies neural developmental disorders such as leukodystrophy. Precise regulation of the activity of specific transcription factors (TFs) by various posttranslational modifications (PTMs) is required to ensure proper oligodendroglial development and myelination. However, the role of ubiquitination of these TFs during oligodendroglial development is yet unexplored. Here, we find that RNF220, a known leukodystrophy-related E3 ubiquitin ligase, is required for oligodendroglial development. RNF220 depletion in oligodendrocyte lineage cells impedes oligodendrocyte progenitor cell proliferation, differentiation, and (re)myelination, which consequently leads to learning and memory defects. Mechanistically, RNF220 targets Olig1/2 for K63-linked polyubiquitination and stabilization during oligodendroglial development. Furthermore, in a knock-in mouse model of leukodystrophy-related RNF220(R365Q) mutation, the ubiquitination and stabilization of Olig proteins are deregulated in oligodendroglial cells. This results in pathomimetic oligodendroglial developmental defects, impaired myelination, and abnormal behaviors. Together, our evidence provides an alternative insight into PTMs of oligodendroglial TFs and how this essential process may be implicated in the etiology of leukodystrophy.
语种英语
WOS研究方向Science & Technology - Other Topics
WOS类目Multidisciplinary Sciences
WOS记录号WOS:001190871400008
来源期刊SCIENCE ADVANCES
文献类型期刊论文
条目标识符http://gcip.llas.ac.cn/handle/2XKMVOVA/292679
作者单位Chinese Academy of Sciences; Kunming Institute of Zoology, CAS; Chinese Academy of Sciences; University of Chinese Academy of Sciences, CAS; Chinese Academy of Sciences; Kunming Institute of Zoology, CAS; Fudan University; Fudan University; Fudan University; Kunming Medical University; Army Medical University; Chinese Academy of Sciences
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Li, Yuwei,Wan, Li Pear,Song, Ning-Ning,et al. RNF220-mediated K63-linked polyubiquitination stabilizes Olig proteins during oligodendroglial development and myelination[J],2024,10(6).
APA Li, Yuwei.,Wan, Li Pear.,Song, Ning-Ning.,Ding, Yu-Qiang.,Zhao, Shuhua.,...&Ma, Pengcheng.(2024).RNF220-mediated K63-linked polyubiquitination stabilizes Olig proteins during oligodendroglial development and myelination.SCIENCE ADVANCES,10(6).
MLA Li, Yuwei,et al."RNF220-mediated K63-linked polyubiquitination stabilizes Olig proteins during oligodendroglial development and myelination".SCIENCE ADVANCES 10.6(2024).
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