Table 1

Patient's OXPHOS activities measured in three different tissues

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)


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.

Schaller et al. BMC Neurology 2011 11:4   doi:10.1186/1471-2377-11-4

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