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

	Activities (mU/U citrate synthase) in
	Skeletal muscle			Fibroblasts
Complex	Patient T	Patient A	Control range	Patient T	Patient A	Control range
I	26	31	70–251	39	39	100–310
II	387	455	335–749	774	544	520–845
III	1306	1870	2200–6610	571	1383	1320–2610
II+III	386	420	300–970	140	218	110–470
IV	322	157	810–3120	102	223	680–1190

Samples were supernatants obtained after 600 g centrifugation of skeletal muscle or mitochondrial-enriched fractions from fibroblasts. The decreased activities as compared with controls are in bold.