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Table 2 Biochemical analysis of patient fibroblasts and muscle biopsies

From: Coenzyme Q10 defects may be associated with a deficiency of Q10-independent mitochondrial respiratory chain complexes

OXPHOS activities (spectrophotometry) I II III IV V G3P + III II + III CS CoQ10 quantity (LC-MSMS) CoQ10
Fibroblast measurements
Control values (nmole/min/mg of proteins) 9.0–27.1 21.0–54.0 62.0–176.2 109.9–350.0 22.0–46.2 6.5–23.0 15.0–37.2 74.7–161.1 Control values (pmole/mg of proteins) 43.0–120.8
P01 11.2 27.7 89.7 29.2 33.5 2.3 7.5 156.2 P01 1.4
P03 11.5 18.5 21.3 177.7 34.1 4.1 12.5 106.7 P03 65.4
P04 7.5 18.6 40.2 108.2 30.5 4.7 8.6 95.0 P04 9.7
P06 11.6 20.6 47.9 78.1 37.6 5.5 10.8 116.6 P06 5.9
P09 12.0 13.4 53.4 57.0 15.8 4.1 8.9 80.9 P09 62.0
P10 13.5 22.9 54.4 65.0 28.3 5.4 12.0 148.2 P10 55.9
P12 10.9 17.6 76.6 173.3 25.0 4.4 10.1 102.5 P12 62.1
P16 11.2 20.7 57.4 134.9 38.3 6.1 14.5 124.0 P16 5.7
P17 12.9 20.0 61.5 181.7 29.3 5.6 14.7 130.3 P17 58.1
P18 14.4 22.5 39.4 78.9 39.3 5.5 13.2 147.0 P18 58.2
Muscle biopsy measurements
Control values (nmole/min/mg of proteins) 11.0–32.0 22.0–65.0 109.0–236.0 93.0-347.0 40.0–89.0 14.0–50.0 20.0–50.0 82.0–234.0 Control values (pmole/mg of proteins) 17.8 –22.2
P02 6.7 21.5 130.4 56.9 32.5 7.4 10.8 113.4 P02 16.0
P05 5.7 21.2 28.9 59.4 12.7 10.9 15.2 122.2 P05 35.5
P07 4.2 28.6 108.8 170.4 50.0 10.5 16.7 272.4 P07 25.9
P08 10.9 14.1 102.5 92.8 63.3 10.8 17.0 116.5 P08 5.7
P11 25.7 29.7 157.6 80.2 45.0 13.6 19.6 109.9 P11 6.2
P13 7.6 28.9 112.7 212.7 58.4 9.4 13.4 192.6 P13 22.4
P14 15.5 26.6 31.6 154.5 32.8 13.7 13.2 100.5 P14 22.2
P15 16.2 20.0 92.3 191.7 39.8 11.9 17.5 86.1 P15 7.1
  1. Respiratory chain enzyme activities were measured spectrophotometrically. Results are expressed as absolute values for controls or patients (in nanomoles of substrate per minute per milligram of protein). CoQ10 quantity was measured by LC-MSMS. Results are expressed as absolute values for controls or patients (in picomoles per milligram of protein). Abnormal values are shown in italics
  2. OXPHOS oxidative phosphorylation; LC-MSMS liquid chromatography coupled with tandem mass spectrometry detection