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DOI | 10.1126/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 |
ISSN | 2375-2548 |
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
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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 |
推荐引用方式 GB/T 7714 | 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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