MTO1-Deficient Mouse Model Mirrors the Human Phenotype Showing Complex I Defect and Cardiomyopathy
Figure 6
Molecular analysis of mouse tissue.
(A) mtDNA copy number in hearts of Mto1−/− mutant mice was significantly reduced as compared to controls (*p<0.05) (B) Respiratory chain complex I was visibly reduced in a Western Blot using a cocktail of OXPHOS antibodies (Mitosciences.com) in Mto1−/− mutant mice as compared to controls while respiratory chain complexes II, II, IV and V were unchanged. (C) Enzymatic activity of complex I in heart tissue lysates was only mildly reduced in Mto1−/− mutant mice but (D) maximal respiratory capacity was significantly reduced in freshly isolated mitochondria (**p<0.01).