Table 1.

Spectrophotometric activity measurements of OXPHOS complexes.

	Skeletal muscle biopsy		Fibroblast		Myoblast
	Patient	Controls	Patient	Controls	Patient	Controls
		(Mean ± SD, n = 25)		(Mean ± SD, n = 8)		(Mean ± SD, n = 4)
Complex I	0.093	0.104	0.195	0.197	0.139	0.151
nmol NADH oxidised·min− 1·unit citrate synthase− 1		± 0.036		± 0.043		± 0.042
Complex II	0.110	0.145	0.187	0.219	0.165	0.126
nmol DCPIP reduced·min− 1·unit citrate synthase− 1		± 0.047		± 0.067		± 0.013
Complex IV	0.318	1.124	1.081	1.083	0.601	1.084
× 10− 3 K s− 1·unit citrate synthase− 1		± 0.511		± 0.186		± 0.105