Table 1.

Citrate synthase-normalized activities of mitochondrial electron transport chain enzyme (ETC) in the patient's skin fibroblasts.

MRC enzyme	Patient	Control (range)
Complex I/CS	0.63	0.86–1.70
Complex II/CS	1.86	1.53–1.87
Complex I + III/CS	0.11	0.55–1.30
Complex II + III/CS	0.08	0.20–0.79
Complex IV/CS	0.54	0.37–1.74
Complex V/CS	8.02	5.07–11.35
Citrate synthase (nmol/min/mg protein)	48.15	49.60–56.18