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Table 1 Mitochondrial respiratory chain enzyme analysis

From: Axial mitochondrial myopathy in a patient with rapidly progressive adult-onset scoliosis

Complex

Electron transport chain activity assay

Patient (nmol/min/mg)

Patient (normalized values)

Control (+/– SD) (nmol/min/mg)1

I

NADH : ferricyanide dehydrogenase

548

154%

280 +/– 91

I + III

NADH : cytochrome C reductase (total)

34.2

86%

28.2 +/– 4.3

NADH : cytochrome C reductase (rotenone sensitive)

5.3

46%

9.1 +/– 2.5

II

Succinate dehydrogenase

12.5

122%

8.1 +/–2.44

II + III

Succinate : cytochrome C reductase

3.19

51%

4.9 +/– 1.1

IV

Sytochrome C oxidase

45.2

122%

29.2 +/– 9.1

None

Citrate synthase (mitochondrial content marker)

355

100%

280 +/– 95

  1. 1Control values for quadriceps femoris (control values for paraspinal muscle are not available).