Article
Modeling of TREX1-Dependent Autoimmune Disease using Human Stem Cells Highlights L1 Accumulation as a Source of Neuroinflammation.
Cell stem cell - 7 Sept 2017
Thomas Charles A, Tejwani Leon, Trujillo Cleber A, Negraes Priscilla D, Herai Roberto H, Mesci Pinar, Macia Angela, Crow Yanick J, Muotri Alysson R
Abstract excerpt
Three-prime repair exonuclease 1 (TREX1) is an anti-viral enzyme that cleaves nucleic acids in the cytosol, preventing accumulation and a subsequent type I interferon-associated inflammatory response. Autoimmune diseases, including Aicardi-Goutières syndrome (AGS) and systemic lupus erythematosus, can arise when TREX1 function is compromised. AGS is a neuroinflammatory disorder with severe and persistent...
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