Article
UBA1-depleted neutrophils disrupt immune homeostasis and induce VEXAS-like autoinflammatory disease in mice.
The Journal of clinical investigation - 3 Nov 2025
Dong Ge, Liu Jingjing, Jin Wenyan, Zhou Hongxi, Wen Yuchen, Wang Zhiqin, Xia Keyao, Zhang Jianlin, Ma Linxiang, Ma Yunxi, Cai Lorie Chen, Zhou Qiufan, Wang Huaquan, Wei Wei, Fu Ying, Cai Zhigang
Abstract excerpt
Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is a hemato-rheumatoid disease caused by somatic UBA1 mutations in hematopoietic stem cells (HSCs). The pathogenic cell type(s) responsible for the syndrome are unknown, and murine models recapitulating the disease are lacking. We report that loss of Uba1 in various mouse hematopoietic cell types resulted in pleiotropic consequences and...
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