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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).