Table 1. .

OXPHOS Enzyme Activities in Skeletal Muscle and Fibroblasts of Patient 1^[Note]

	Activities (mU/U citrate synthase) in
	Skeletal Muscle		Fibroblasts
Complex	Patient	Reference Range	Patient	Reference Range
I	0	70–250	53	100–310
III	753	2,200–6,610	1,250	1,320–2,610
II + III	228	300–970	163	110–470
IV	186	810–3,120	397	680–1,190
PDH	60	34–122	ND	ND

Note.— Samples were 600 g of supernatant of skeletal muscle or mitochondrial enriched fraction from fibroblasts. ND = not determined; PDH= pyruvate dehydrogenase complex.