Table 1.

Respiratory-Chain Activities in Liver Homogenate, Muscle Mitochondria, and Fibroblasts

	Liver Homogenate		Muscle Mitochondria		Fibroblasts
	Subject 1	Control	Subject 1	Control	Subject 2	Control
Absolute Activity (nmol/min/mg of Protein)
CI	20	25 ± 5	108	73 ± 15	14	13 ± 3
CII	140	146 ± 20	143	98 ± 20	28	21 ± 2
CIII	185	400 ± 60	2,343	1,458 ± 257	247	181 ± 20
CIV	48	187 ± 29	1,073	740 ± 146	132	103 ± 11
CV	149	112 ± 29	539	335 ± 68	61	65 ± 6
CS	48	61 ± 9	573	399 ± 70	75	75 ± 7
Activity Ratios
CI/CS	0.41	0.41 ± 0.08	0.19	0.18 ± 0.02	0.19	0.17 ± 0.03
CII/CS	2.93	2.23 ± 0.30	0.25	0.25 ± 0.02	0.38	0.29 ± 0.02
CIII/CS	3.86	6.43 ± 0.85	4.09	3.91 ± 0.40	3.31	2.53 ± 0.20
CIV/CS	0.99	3.03 ± 0.38	1.87	1.96 ± 0.15	1.77	1.35 ± 0.11
CV/CS	3.11	1.80 ± 0.34	0.94	0.80 ± 0.06	0.27	0.27 ± 0.03

Abnormal values are in bold. The following abbreviations are used: CI–CV, complexes I–V; and CS, citrate synthase.