DNA damage contributes to neurotoxic inflammation in Aicardi-Goutières syndrome astrocytes

A. Giordano, M. Luciani, F. Gatto, Monah Abou Alezz, Chiara Beghè, Lucrezia Della Volpe, A. Migliara, Sara Valsoni, M. Genua, M. Dzieciatkowska, Giacomo Frati, Julie Tahraoui-Bories, S. Giliani, S. Orcesi, E. Fazzi, Renato Ostuni, Angelo D’Alessandro, Raffaella Di Micco, I. Merelli, A. Lombardo, Martin A. M. Reijns, N. Gromak, A. Gritti, A. Kajaste-Rudnitski
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引用次数: 25

Abstract

Giordano et al. establish human AGS iPSC-based models of TREX1 or RNASEH2B deficiencies. They uncover DNA damage as a major driver of neurotoxic inflammation in AGS astrocytes that can be uncoupled from aberrant type I IFN responses and identify novel targets to curtail AGS neuroinflammation.
DNA损伤有助于aicardii - gouti综合征星形胶质细胞的神经毒性炎症
Giordano等人建立了TREX1或RNASEH2B缺陷的人类AGS ipsc模型。他们发现DNA损伤是AGS星形胶质细胞神经毒性炎症的主要驱动因素,可以从异常的I型IFN反应中解耦,并确定了减少AGS神经炎症的新靶点。
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