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Table 2 The activities of respiratory chain complexes in isolated muscle mitochondria

From: Multisystem mitochondrial diseases due to mutations in mtDNA-encoded subunits of complex I

PatientIndividual respiratory chain complexes activities in isolated muscle mitochondria
CI/CSCI + III/CSCII/CSCII + III/CSCIII/ CSCIV/CS
patientage related control rangepatientage related control rangepatientage related control rangepatientage related control rangepatientage related control rangepatientage related control range
10.500.45–1.050.180.17–0.310.090.07–0.270.330.17–0.470.430.27–0.851.591.1–2.22
30.130.18–0.380.080.18–0.370.040.05–0.110.240.17–0.320.670.46–0.881.210.66–2.25
40.240.45–1.050.060.17–0.310.090.07–0.270.340.17–0.470.950.27–0.850.971.1–2.22
50.110.15–0.410.010.13–0.250.050.05–0.110.230.15–0.270.720.3–0.561.50.66–2.25
60.060.45–1.050.080.17–0.310.070.07–0.270.260.17–0.470.720.27–0.851.121.1–2.22
70.080.18–0.380.060.18–0.370.070.05–0.110.380.17–0.320.740.46–0.881.111.16–2.13
80.160.15–0.410.050.13–0.250.060.05–0.110.440.15–0.271.70.3–0.561.120.66–2.25
90.280.18–0.380.030.18–0.370.090.05–0.110.380.17–0.320.660.46–0.881.271.16–2.13
100.110.18–0.380.080.18–0.370.050.05–0.110.300.17–0.320.570.46–0.881.71.16–2.13
110.070.18–0.380.030.18–0.370.040.05–0.110.190.17–0.320.520.46–0.880.961.16–2.13
  1. The reference ranges for individual patients according to age of patients are displayed in next column. Controls are constituted in the three major groups (0–2 years old; 3–18 years old and adults). Alterations in patient’s complexes activities are shown in bold. Abbreviations: CI complex I, NADH coenzyme Q reductase; CI + III complex I + III, NADH cytochrome c reductase; CII complex II, succinate:coenzyme Q reductase; CII + III complex II + III, succinate:cytochrome c oxidoreductase; CIII complex III, coenzyme Q:cytochrome c oxidoreductase; CIV complex IV, cytochrome c oxidase; CS citrate synthase