Table 1. OXPHOS enzyme activities in fibroblasts.

	Activities (mU/U citrate synthase)a,b
Complex	Patient	Reference range
I	45	100–310
II	606	520–845
III	782	1320–2610
II+III	186	110–470
IV	242	680–1190

^aMeasured in mitochondrial-enriched fractions from fibroblasts.

^bThe activity of citrate synthase was 208 mU/mg protein (reference range 144–257 mU/mg protein).

Decreased activities as compared with controls are shown in bold numbers.