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
{"title":"DNA damage contributes to neurotoxic inflammation in Aicardi-Goutières syndrome astrocytes","authors":"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","doi":"10.1084/jem.20211121","DOIUrl":null,"url":null,"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.","PeriodicalId":23015,"journal":{"name":"The Tokushima journal of experimental medicine","volume":null,"pages":null},"PeriodicalIF":0.0000,"publicationDate":"2022-03-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"25","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"The Tokushima journal of experimental medicine","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1084/jem.20211121","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
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.