TABLE 1.

Biochemical analysis of mitochondrial respiratory chain complexes activities.

	Activities (nmol.min–1.mg–1)	Ref. range (nmol.min–1.mg–1)
	Patient
CI	20.91	[10.42–47.30]
CII	54.36*	[18.73–47.70]
CIII	121.97	[81.28–210.86]
CIV	264.79*	[82.01–237.59]
CV	105.28	[62.21–130.72]
CII+III	45.79*	[16.21–33.24]
CS	226.23	[110.86–288.65]
LDH	5448	[2084–7317]

Spectrophotometry-based assays were used in order to analyze isolated enzymatic activities of NADH dehydrogenase (CI), succinate dehydrogenase (CII), coenzyme Q cytochrome c-oxidoreductase (CIII), cytochrome c oxidase (CIV) and ATP synthase (CV) as well as combined CII+III activities. Citrate synthase (CS) activity was used as a control for mitochondrial mass, while lactate dehydrogenase (LDH) was used as a cytosolic reference. Normalized activities, expressed in terms of the citrate synthase level for each individual complex were within normal ranges in both cases. Values represent enzymatic activities and are expressed in nanomoles of substrate transformed per minute per milligrams of proteins (nmol.min^–1.mg^–1). *indicates activities that do not fall within our normal reference values.