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Table 1 Patient's OXPHOS activities measured in three different tissues

From: Molecular and biochemical characterisation of a novel mutation in POLGassociated with Alpers syndrome

  Fibroblasts Skeletal muscle homogenate Liver homogenate
Enzymes Patient Controls (n = 22) Patient Controls (n = 26) Patient Controls (n = 12)
Complex I 0.29 0.19 - 0.46 (0.29 +/- 0.07) 0.16/0.13* 0.12 - 0.28 (0.19 +/- 0.04) 0.007/0.008* 0.22 - 0.76 (0.43 +/- 0.2)
Complex II 0.25 0.17 - 0.52 (0.33 +/- 0.09) 0.13/0.15* 0.14 - 0.36 (0.21 +/- 0.05) 0.68/0.75* 0.59 - 2.11 (1.35 +/- 0.45)
Complex III 0.42 0.35 - 0.87 (0.6 +/- 0.15) 0.74 0.55 - 1.11 (1.16 +/- 0.28) 0.26/0.3* 0.54 - 2.16 (1.47 +/- 0.49)
Complex IV 0.49 0.42 - 1.11 (0.75 +/- 0.18) 0.27/0.17* 0.57 - 1.77 (0.78 +/- 0.15) 0.35/0.42* 0.74 - 5.17 (2.1 +/- 1.2)
Complex V 0.16 0.14 - 0.42 (0.22 +/- 0.08) 0.19 0.19 - 0.65 (0.39 +/- 0.13) 0.42 0.25 - 1.14 (0.58 +/- 0.28)
Citrate synthase 172 106 - 317 (184 +/- 43) 124 70 - 169 (105 +/- 25) 68 21 - 40 (31 +/- 6.5)
  1. Activities of the respiratory chain complexes are normalised to citrate synthase (CS) and are expressed as mU/mg mitochondrial protein. Mean value (+/- 1SD) of controls are indicated in brackets. Profound deficiencies below the range of controls are shown in bold. n = number of controls; * = measured as duplicate.
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BMC Neurology

ISSN: 1471-2377

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