Table 3.
Results of enzyme activity measurements in fibroblast cell lines. The numbering of the cell lines is given in Table 2.
| Complex I |
Complex II |
Complex III |
Complex IV |
|||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Lab 1 | Lab 2 | Lab 3 | Lab 4 | Lab 1 | Lab 2 | Lab 3 | Lab 4 | Lab 1 | Lab 2 | Lab 3 | Lab 4 | Lab 1 | Lab 2 | Lab 3 | Lab 4 | |
| F1 | 57.0 | 17.7 | 4.0 | 38.8 | 99.0 | 16.8 | 14.5 | 134.6 | 841.0 | 133.6 | 10.5 | 333.0 | 411.0 | 126.1 | 28.4 | 197.0 |
| F2 | 75.0 | 27.1 | 12.3 | 38.4 | 118.0 | 22.0 | 12.1 | 210.9 | 1235.0 | 168.6 | 24.5 | 456.0 | 160.0 | 42.4 | 9.7 | 71.0 |
| F3 | 55.0 | 12.6 | 12.4 | 47.4 | 88.0 | 10.6 | 4.6 | 191.7 | 780.0 | 97.7 | 13.4 | 485.0 | 419.0 | 94.4 | 38.8 | 240.0 |
| F4 | 59.0 | 11.9 | 13.2 | 27.2 | 92.0 | 8.3 | 8.6 | 150.6 | 696.0 | 64.8 | 39.2 | 346.0 | 424.0 | 70.6 | 53.0 | 193.0 |
| F5 | 58.0 | 15.7 | 7.0 | 26.7 | 105.0 | 12.4 | 7.6 | 141.4 | 640.0 | 83.8 | 12.1 | 250.0 | 452.0 | 79.5 | 20.0 | 178.0 |
| F7 | 62.0 | 22.7 | 12.3 | 30.9 | 103.0 | 12.7 | 4.5 | 171.2 | 793.0 | 66.9 | 20.7 | 411.0 | 496.0 | 83.0 | 33.9 | 216.0 |
| F8 | 51.0 | 17.3 | 7.7 | 39.4 | 33.0 | 4.0 | 3.7 | 42.6 | 574.0 | 71.2 | 11.9 | 310.0 | 335.0 | 82.8 | 16.2 | 176.0 |
| F10 | 55.0 | 20.7 | 4.9 | 49.0 | 91.0 | 14.3 | 6.5 | 247.4 | 532.0 | 75.1 | 22.5 | 547.0 | 357.0 | 93.8 | 34.0 | 280.0 |
| FA-P1 | 23.0 | 2.5 | 0.1 | 6.1 | 186.0 | 17.1 | 4.3 | 111.3 | 1405.0 | 70.1 | 8.9 | 313.0 | 1025.0 | 81.1 | 24.0 | 149.0 |
| FA-P2 | 54.0 | 4.9 | 4.1 | 9.0 | 174.0 | 17.6 | 21.0 | 137.0 | 1084.0 | 65.4 | 30.0 | 247.0 | 274.0 | 32.8 | 20.0 | 21.0 |
| 99RD0204 | 46.0 | 12.5 | 6.2 | 23.0 | 79.0 | 11.4 | 7.9 | 107.8 | 931.0 | 126.6 | 14.0 | 235.0 | 323.0 | 104.7 | 28.4 | 136.0 |
| 00RD0395 | 44.0 | 5.3 | 4.8 | 12.5 | 112.0 | 12.3 | 9.1 | 153.3 | 969.0 | 86.1 | 20.0 | 377.0 | 469.0 | 87.2 | 36.0 | 219.0 |
| 04RD0504 | 38.0 | 16.6 | 7.0 | 16.4 | 43.0 | 18.7 | 11.1 | 101.3 | 667.0 | 63.9 | 28.4 | 241.0 | 112.0 | 34.6 | 8.7 | 74.0 |
| I | 48.0 | n.d. | 17.5 | 23.3 | 107.0 | n.d. | 14.5 | 121.3 | 1852.0 | n.d. | 57.0 | 465.0 | 107.0 | n.d. | 15.0 | 16.0 |
| Ib | 45.0 | n.d. | 12.9 | 17.9 | 73.0 | n.d. | 11.7 | 116.8 | 1090.0 | n.d. | 48.0 | 289.0 | 45.0 | n.d. | 8.2 | 14.0 |
| III | 38.0 | n.d. | 6.3 | 2.3 | 83.0 | n.d. | 20.0 | 95.6 | 1102.0 | n.d. | 43.0 | 188.0 | 158.0 | n.d. | 31.0 | 3.0 |
| Complex V |
Citrate synthase |
Complex II + III |
||||||||
|---|---|---|---|---|---|---|---|---|---|---|
| Lab 1 | Lab 2 | Lab 3 | Lab 4 | Lab 1 | Lab 2 | Lab 3 | Lab 4 | Lab 1 | Lab 4 | |
| F1 | 159.0 | 98.0 | 11.8 | 168.0 | 146.0 | 116.0 | 49.8 | 177.0 | 97.0 | 49.0 |
| F2 | 237.0 | 132.4 | 14.8 | 207.0 | 240.0 | 151.6 | 68.2 | 285.0 | 109.0 | 85.0 |
| F3 | 36.0 | 36.9 | 6.1 | 12.0 | 181.0 | 93.0 | 50.9 | 275.0 | 97.0 | 64.0 |
| F4 | 118.0 | 67.2 | 12.9 | 170.0 | 128.0 | 59.6 | 60.3 | 185.0 | 60.0 | 59.0 |
| F5 | 130.0 | 76.1 | 15.7 | 124.0 | 134.0 | 75.4 | 36.2 | 159.0 | 72.0 | 49.0 |
| F7 | 130.0 | 55.1 | 15.9 | 192.0 | 155.0 | 57.6 | 38.2 | 208.0 | 92.0 | 60.0 |
| F8 | 143.0 | 111.5 | 15.3 | 178.0 | n.d. | 76.1 | 57.3 | 162.0 | 23.0 | 24.0 |
| F10 | 127.0 | 89.4 | 17.1 | 255.0 | n.d. | 75.8 | 51.2 | 278.0 | 63.0 | 92.0 |
| FA-P1 | 470.0 | 60.1 | 15.0 | 66.0 | 311.0 | 68.8 | 42.4 | 146.0 | 294.0 | 38.0 |
| FA-P2 | 189.0 | 69.0 | 17.0 | 78.0 | 329.0 | 79.9 | 84.0 | 185.0 | 254.0 | 50.0 |
| 99RD0204 | 54.0 | 39.7 | 8.2 | 42.0 | 184.0 | 93.0 | 47.0 | 115.0 | 137.0 | 31.0 |
| 00RD0395 | 129.0 | 89.9 | 17.4 | 173.0 | 203.0 | 84.5 | 104.0 | 172.0 | 161.0 | 76.0 |
| 04RD0504 | 105.0 | 77.1 | 18.8 | 158.0 | 125.0 | 76.4 | 87.0 | 198.0 | 85.0 | 35.0 |
| I | 146.0 | n.d. | 19.0 | 99.0 | 337.0 | n.d. | 103.0 | 228.0 | 187.0 | 62.0 |
| Ib | 88.0 | n.d. | 14.0 | 79.0 | 244.0 | n.d. | 64.5 | 170.0 | 165.0 | 41.0 |
| III | 138.0 | n.d. | 22.0 | 131.0 | 291.0 | n.d. | 98.0 | 159.0 | 201.0 | 29.0 |
Activities are in nmoles per min per mg protein for lab 1 and in units per mg protein for labs 2–4.