Article
RNase H2 Loss in Murine Astrocytes Results in Cellular Defects Reminiscent of Nucleic Acid-Mediated Autoinflammation.
Frontiers in immunology - 1 Jan 2018
Bartsch Kareen, Damme Markus, Regen Tommy, Becker Lore, Garrett Lillian, Hölter Sabine M, Knittler Katharina, Borowski Christopher, Waisman Ari, Glatzel Markus, Fuchs Helmut, Gailus-Durner Valerie, Hrabe de Angelis Martin, Rabe Björn
Abstract excerpt
Aicardi-Goutières syndrome (AGS) is a rare early onset childhood encephalopathy caused by persistent neuroinflammation of autoimmune origin. AGS is a genetic disorder and >50% of affected individuals bear hypomorphic mutations in ribonuclease H2 (RNase H2). All available RNase H2 mouse models so far fail to mimic the prominent CNS involvement seen in AGS. To establish a mouse model recapitulating the human...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.