Microglia replacement halts the progression of microgliopathy in mice and humans.

doi:10.1126/science.adr1015

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Article

Microglia replacement halts the progression of microgliopathy in mice and humans.

Science (New York, N.Y.) - 10 Jul 2025

Wu Jingying, Wang Yafei, Li Xiaoyu, Ouyang Pei, Cai Yuanyuan, He Yang, Zhang Mengyuan, Luan Xinghua, Jin Yuxiao, Wang Jie, Xiao Yujie, Liang Yuqing, Xie Fang, Shu Yousheng, Hu Jiong, Chang Chunkang, Jiang Jieling, Wu Dong, Zhao Youshan, Liu Taohui, Li Yuxin, Huang Xiaojun, Li Yao, Zhang Junfang, Cao Yuwen, Cheng Xin, Mao Ying, Rao Yanxia, Cao Li, Peng Bo

Abstract excerpt

Colony-stimulating factor 1 receptor (CSF1R) is primarily expressed in microglia. Its monoallelic mutation causes CSF1R-associated microgliopathy (CAMP), a major form of adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) and a fatal neurological disease without clinical cure. We developed mouse models harboring human hotspot mutations of CAMP and replaced CSF1R-deficient microglia...

Read the complete abstract on PubMed

Topics

Microglia
Animals
Humans
Mice
Disease Progression

Methodology Data sources Editorial policy Privacy Delete account & data About Contact Helena Bioinformatics

Receptors, Granulocyte-Macrophage Colony-Stimulating Factor

Bone Marrow Transplantation

Disease Models, Animal

Mutation

Leukoencephalopathies

Female

Male

Receptor, Macrophage Colony-Stimulating Factor

Source

Open in PubMed

Identifiers

PMID: 40638739
DOI: 10.1126/science.adr1015

Genes

CSF1R

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 24 Oct 2026.

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