Published in

American Association for the Advancement of Science, Science Advances, 43(8), 2022

DOI: 10.1126/sciadv.abo5840

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ATR-mediated DNA damage responses underlie aberrant B cell activity in systemic lupus erythematosus

This paper is made freely available by the publisher.
This paper is made freely available by the publisher.

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Abstract

B cells orchestrate autoimmune responses in patients with systemic lupus erythematosus (SLE), but broad-based B cell–directed therapies show only modest efficacy while blunting humoral immune responses to vaccines and inducing immunosuppression. Development of more effective therapies targeting pathogenic clones is a currently unmet need. Here, we demonstrate enhanced activation of the ATR/Chk1 pathway of the DNA damage response (DDR) in B cells of patients with active SLE disease. Treatment of B cells with type I IFN, a key driver of immunity in SLE, induced expression of ATR via binding of interferon regulatory factor 1 to its gene promoter. Pharmacologic targeting of ATR in B cells, via a specific inhibitor (VE-822), attenuated their immunogenic profile, including proinflammatory cytokine secretion, plasmablast formation, and antibody production. Together, these findings identify the ATR-mediated DDR axis as the orchestrator of the type I IFN–mediated B cell responses in SLE and as a potential novel therapeutic target.