Table 1.

Isolated complex IV deficiency in muscle biopsies of subject 1 and subject 2

	CI	CII	CIII	CIV	CV	CS
Subject 1	298	456	947	123	719	475
Subject 2	324	412	721	103	809	283
Reference range	163–599	335–888	570–1383	288–954	193–819	151-449

Activities of all complex activities are expressed as milliunits per unit citrate synthase (mU/U CS). The activity of CS is expressed as milliunits per milligram protein (mU/mg). Respiratory chain complex activities below the reference range are indicated in bold