Author response: Microglia replacement by ER-Hoxb8 conditionally immortalized macrophages provides insight into Aicardi–Goutières syndrome neuropathology

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Authors: Kelsey Marie Nemec, Genevieve Gucci Grace Capispisan Uy, V Sai Chaluvadi, Freddy S Purnell, Bilal Elfayoumi, Leila Byerly, Micaela L. O’Reilly, Carleigh A. O’Brien, William H. Aisenberg, Sonia I Lombroso, Xinfeng Guo, Niklas Blank
+10 more Chet Huan Oon, Fazeela Yaqoob, Brian Temsamrit, Priyanka Rawat, Christoph A. Thaiss, Will Bailis, Adam P. Williamson, Qingde Wang, Mariko L. Bennett, F. Chris Bennett
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Abstract

A microglia replacement approach demonstrates that brain macrophages with patient mutations from Aicardi–Goutières syndrome, a genetic, brain predominant interferonopathy, are sufficient to drive interferon responses.

Fields of Study & Subjects

Neuroinflammation and Neurodegeneration Mechanisms interferon and immune responses Inflammasome and immune disorders

Publication details

Publication date
27 January 2026