Table 1. OXPHOS enzyme activities in skeletal muscle and skin fibroblasts.

	Activities (mU/U citrate synthase) in
	Skeletal muscle		Skin fibroblasts
Complex	Patient	Reference range	Patient	Reference range
I	79	70–250	34	100–310
II	97	67–177	595	520–845
III	2151	2200–6610	1038	1320–2610
II+III	319	300–970	122	110–470
IV	1103	810–3120	168	680–1190

Abbreviation: OXPHOS, oxidative phosphorylation.

Samples were 600 g of supernatant of skeletal muscle or mitochondrial enriched fraction from fibroblasts.

Decreased activities as compared with controls are in bold.