Cosmc deficiency causes spontaneous autoimmunity by breaking B cell tolerance.
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ABSTRACT:
SUBMITTER: Zeng J
PROVIDER: S-EPMC8494437 | biostudies-literature | 2021 Oct
REPOSITORIES: biostudies-literature
Publications
<i>Cosmc</i> deficiency causes spontaneous autoimmunity by breaking B cell tolerance.
Zeng Junwei J Aryal Rajindra P RP Stavenhagen Kathrin K Luo Chi C Liu Renyan R Wang Xiaohui X Chen Jiaxuan J Li Hao H Matsumoto Yasuyuki Y Wang Yingchun Y Wang Jianmei J Ju Tongzhong T Cummings Richard D RD
Science advances 20211006 41
Factors regulating the induction and development of B cell–mediated autoimmunity are not well understood. Here, we report that targeted deletion in murine B cells of X-linked <i>Cosmc</i>, encoding the chaperone required for expression of core 1 O-glycans, causes the spontaneous development of autoimmune pathologies due to a breakdown of B cell tolerance. BC-<i>Cosmc</i>KO mice display multiple phenotypic abnormalities, including severe weight loss, ocular manifestations, lymphadenopathy, and in ...[more]
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