Table 1

Enzymological investigation of skeletal muscle homogenates and cultures of skin fibroblasts from patient and controls

		Activities (nmol/min/mg protein)
		Patient	Control (n=51)
Muscle homogenate
CI	31	8–35
CII	37	11–65
CIII	176	44–227
CIV	45	65–330
CV	96	29–162
CII+CIII	40	12–59
	Patient	Control (n=85)
Fibroblasts
CII	25	12–42
CIII	118	33–187
CIV	21	47–182
GPDH	17	6–26
CII+CIII	38	16–68
	Activity ratios
	Patient	Control (n=51)
Muscle homogenate
CIV/CI	1.5	10.4±1.7
CIV/CII	1.2	5.7±0.6
CIV/CIII	0.3	1.4±0.2
CIV/CV	0.5	2.1±0.3
	Patient	Control (n=85)
Fibroblasts
CIV/CII	0.8	4.7±0.7
CIV/CIII	0.2	1.0±0.2
CII/GPDH	1.5	1.6±0.2

CI-V: the various complexes of the respiratory chain. Abnormal values appear in bold.