. Author manuscript; available in PMC: 2022 Jun 23.

Published in final edited form as: J Inherit Metab Dis. 2021 Apr 27;44(4):949–960. doi: 10.1002/jimd.12387

TABLE 1.

Clinical results of electron transport chain activities in skeletal muscle biopsy by Baylor Miraca Genetics Laboratory

Electron transport chain activities	ETC complexes	Patient (% of mean) (nmol min⁻¹ mg⁻¹ protein)	Control ± SD (nmol min⁻¹ mg⁻¹ protein)
NADH:Ferricyanide dehydrogenase	I	78 (22) low	360.4 ± 96.3
NADH:cytochrome c reductase	I + III
Total		16.1 (57)	28.4 ± 6.1
Rotenone sensitive		0.94 (11) low	8.7 ± 3.9
Succinate dehydrogenase	II	7.17 (75)	9.6 ± 3.0
Succinate: cytochrome c reductase	II + III	3.3 (79)	4.2 ± 1.2
Cytochrome c oxidase	IV	10.4 (26) low	40.3 ± 15.5
Citrate synthase		320 (109)	293.1 ± 68.0