Inhibition of STING Protects the Neuroretina in an Ndufs4 Knockout Mouse Model of Mitochondrial Complex 1 Deficiency.
PURPOSE Ndufs4 encodes a key subunit of mitochondrial complex I, and its mutation causes NADH dehydrogenase deficiency associated with Leigh syndrome and Leber hereditary optic neuropathy. In Ndufs4 knockout (KO) mice, vision loss occurs alongside an "inflammatory wave" that disrupts neuroretinal function. In this stud...