Table 2.

Respiratory Chain Activities Referred to the Activity of the Matrix Enzyme CS in Muscle Extracts of the Patient and of 69 Control Subjects

	nmol/min per CS Units
Enzyme (Complex)	Controls, Mean (SD)	Muscle Extracts of the Patient (% of Controls)
NADH–cytochrome-c reductase (I plus III)	103 (38)	73 (71)
Succinate–cytochrome-c reductase (II plus III)	71 (20)	70 (99)
Cytochrome-c oxidase (IV)	283 (50)	238 (84)
Succinate dehydrogenase (II)	101 (31)	107 (106)

Abbreviations: CS, citrate synthase; NADH, nicotinamide adenine dinucleotide.