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. 2010 Jun 16;18(10):1160–1165. doi: 10.1038/ejhg.2010.83

Table 2. Activities of the mitochondrial respiratory chain complexes in the muscle and myocardium.

  Sample
  Muscle mitochondria Myocardium homogenate
Assay Control muscle mitochondria n=50 B3 A5 Control myocardium n=3 A8 A5
Citrate synthasea 1990±370 1690 1800 251±164 454 233
NADH-CoQ reductasea (complex I) 241±92 149 (74%) 110 (50%)      
NADH-cytochrome c reductasea (complex I+III) 586±234 413 (84%) 335 (73%)      
Succinate – cytochrome c reductasea (complex II+III) 312±118 133 (51%) 115 (64%) 27±13 10 (21%) 2 (8%)
Succinate-CoQ reductasea (complex II) 72±26 30 (50%) 39 (60%)      
Succinate dehydrogenasea (complex II) 260±86 118 (54%) 131 (56%) 48±25 13 (15%) 8 (18%)
Ubiquinol-cytochrome c reductasea (complex III) 4270±970 3800 (106%) 3000 (99%)      
Cytochrome c oxidasea (complex IV) 1155±420 1135 (117%) 1128 (108%) 197±118 354 (100%) 147 (81%)
Mg++ATPase (complex V)a 536±193 828 (184%) 675 (111%)      
Pyruvate+malate oxidationb 67±32 59 (105%)        
Succinate oxidationb 94±36 20 (26%)        

The assignment of patients is according to Figure 1.

a

Activities of the five mitochondrial respiratory chain complexes were measured spectrophotometrically in nmol/min per mg.

b

Activities of the five mitochondrial respiratory chain complexes were measured by oxygen consumption in nat O/min per mg protein.

Values in brackets present activities as a percentage of the control mean, normalized for citrate synthase activity.