Table 2.

Enzymatic activities of the mitochondrial respiratory chain in the fibroblasts from C9ALS/FTD patients.

Enzyme Activities 1	Fibroblasts
	C9ORF72_1	C9ORF72_2	C9ORF72_3	C9ORF72_4	Control Range
Complex I+III	17.4	9.6 *	0 *	25.6	15–42
Complex II	19.2 *	34	18.1 *	40.6	22–35
Complex II+III	3.4 *	9.8 *	1.8 *	15.8	11–20
Complex III	4.6 *	8.3 *	0 *	14.2 *	25–48
Complex IV	67	42.2	18.1 *	68.5	33–57
Citrate synthetase (CS)	18.7 *	54	15.8 *	61.8	41–62
Enzyme Activities 2	C9ORF72_1	C9ORF72_2	C9ORF72_3	C9ORF72_4	Control Range
Complex I+III/CS	0.930	0.178 *	0 *	0.414	0.24–0.85
Complex II/CS	1.027	0.630	1.146	0.657	0.53–0.75
Complex II+III/CS	0.182 *	0.181 *	0.114 *	0.256	0.25–0.42
Complex III/CS	0.246 *	0.154 *	0 *	0.230 *	0.49–1.06
Complex IV/CS	3.583	0.781	1.146	1.108	0.84–1.15

¹ nmol min⁻¹ mg⁻¹ protein; ² nmol min⁻¹ mg⁻¹ protein enzyme activity expressed in percentage respect to CS activity; * Values below the control range.