Prion-like Aggregation of Mitochondrial Antiviral Signaling Protein in Lupus Patients Is Associated With Increased Levels of Type I Interferon: MAVS AGGREGATION AND TYPE I IFN IN LUPUS
Increased levels of Type I interferon (IFN-I) and IFN-I-regulated genes are found in patients with systemic lupus erythematosus (SLE) and may be central to its pathogenesis. The mitochondrial adaptor protein MAVS is a key regulator of IFN-I that undergoes a dramatic prion-like aggregation and self-propagates the activation signal from viral RNA to amplify downstream IFN production. We wondered if such MAVS aggregates might play a role in the sustained increased production of IFN-I in SLE
