Microglia loss triggers glial stress and white matter damage in human leukodystrophy

IF 27.6 1区医学 Q1 IMMUNOLOGY

Nature Immunology Pub Date : 2025-06-26 DOI:10.1038/s41590-025-02194-8

引用次数: 0

Abstract

We show that mutations in the CSF1R gene, which cause the rare neurodegenerative disease ALSP, lead to the loss and abnormal activation of microglia. This triggers glial stress and the emergence of disease-associated oligodendrocytes with impaired myelinating potential. These findings suggest potential therapeutic strategies by modulating glial stress pathways or enhancing compensatory microglial support mechanisms.

Abstract Image

查看原文本刊更多论文

在人类脑白质营养不良中，小胶质细胞的丢失会引发胶质细胞应激和白质损伤

我们发现，导致罕见神经退行性疾病ALSP的CSF1R基因突变导致小胶质细胞的丢失和异常激活。这触发神经胶质应激和疾病相关的少突胶质细胞的出现，髓鞘潜能受损。这些发现提示了通过调节神经胶质应激途径或增强代偿性小胶质细胞支持机制的潜在治疗策略。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

求助全文

约1分钟内获得全文求助全文

来源期刊

Nature Immunology 医学-免疫学

CiteScore

40.00

自引率

2.30%

发文量

248

审稿时长

4-8 weeks

期刊介绍： Nature Immunology is a monthly journal that publishes the highest quality research in all areas of immunology. The editorial decisions are made by a team of full-time professional editors. The journal prioritizes work that provides translational and/or fundamental insight into the workings of the immune system. It covers a wide range of topics including innate immunity and inflammation, development, immune receptors, signaling and apoptosis, antigen presentation, gene regulation and recombination, cellular and systemic immunity, vaccines, immune tolerance, autoimmunity, tumor immunology, and microbial immunopathology. In addition to publishing significant original research, Nature Immunology also includes comments, News and Views, research highlights, matters arising from readers, and reviews of the literature. The journal serves as a major conduit of top-quality information for the immunology community.