Ocular Pathology in Mice Lacking Mitochondrial Manganese Superoxide Dismutase

Mitochondrial superoxide dismutase (SOD) is an important enzyme for disposal of reactiveoxygen species generated during oxidative phosphorylation. Genetic inactivation of SOD2 in mice causes dilated cardiomyopathy, hepatic lipid accumulation and neonatal death. Treatment with the SOD mimetic Manganese 5,10,15,20-tetrakis (4-benzoic acid) porphyrin (MnTBAP), which does not cross the blood brain barrier, doubles survival time, but the animals develop a pronounced movement disorder and spongiform encephalopathy. If the retina, another highly metabolically active neural tissue is also affected in the SOD2 deficient mouse, then this could provide a useful animal model for human mitochondrial disorders affecting the eye.