
A catalytic defect in mitochondrial respiratory chain complex I due to a mutation in NDUFS2 in a patient with Leigh syndrome
Keywords: زنجیر تنفسی; BN-PAGE, blue native-PAGE; CoQ, coenzyme Q; CS, citrate synthase; OXPHOS, oxidative phosphorylationComplex I; NDUFS2; Mitochondrion; Respiratory chain; Enzyme activity; Coenzyme Q