MIM-IR: Characterization of the links between inner mitochondrial membrane homeostasis and interferon signaling. 2025-2027, ANR, PI Sylvie Bannwarth
Mitochondrial cristae disorganization can lead to mitochondrial nucleic acid release into cytosol inducing an up-regulation of the type I interferon (IFN) signaling via the cGAS-STING pathway. Thus, some mitochondrial diseases may be considered as an autoinflammatory disease (type I interferonopathy) and neurological signs may be caused by this inflammation.
Our project aims to determine how the interferon signaling contributes to pathophysiology in patients with mitochondrial disease.
We will characterize (i) key partners responsible of inner mitochondrial membrane maintenance, (ii) stress responses to inner mitochondrial membrane disorganization and (iii) the role of Sting in the neurological phenotype of KI Chchd10S59L/+ mouse.