Table 1.

Enzymatic activities of mitochondrial respiratory chain complexes

Assaya	Muscle mitochondria		Fibroblast homogenate
	Patient	Controls ± SD (range) n = 50	Patient	Control mean n = 4
CI	106 [98%]	98 ± 46 (46–215)	nd	nd
CI + III	159 [65%]	305 ± 148 (104–717)	nd	nd
CII	141 [116%]	124 ± 57 (52–230)	nd	nd
CII + III	75 [105%]	80 ± 40 (41–172)	nd	nd
CIV	209 [25%]	1,061 ± 47 (265–2,896)	61 [115%]	72 ± 22 (62–135)
CS	980	1,132 ± 438 (438–2,350)	42	45 ± 12 (30–66)

nd not determined

^anmol/min/mg (%) percentage residual activity normalized to citrate synthase (CS) activity compared to controls