Table 1.

Respiration rates in untreated and dNs treated control and ECHS1 KO cells.

Substrate		Basal pmol O2/s/mg	Maximal pmol O2/s/mg	Proton Leak pmol O2/s/mg	Spare Respiratory Capacity	Cell Respiratory Control Ratio
Palmitoyl-L-Carnitine	CON UT	157 ± 37	326 ± 74	65 ± 14	169 ± 38	5 ± 1
	CON + dNs	917 ± 197 **	2230 ± 93 ***	56 ± 10	1312 ± 290 ***	40 ± 2 ****
	ECHS1 KO UT	98 ± 30 *	148 ± 37 **	67 ± 10	51 ± 13 **	2 ± 1 **
	ECHS1 KO + dNs	612 ± 336 #	1036 ± 532 #	74 ± 6	424 ± 264 #	14 ± 7 #
Glucose	CON UT	160 ± 17	891 ± 86	132 ± 28	726 ± 74	7 ± 1
	CON + dNs	165 ± 49	1031 ± 97	53 ± 35 *	851 ± 75	31 ± 27
	ECHS1 KO UT	111 ± 7 ***	513 ± 16 ***	88 ± 54	404 ± 19 ****	9 ± 9
	ECHS1 KO + dNs	180 ± 59 #	1065 ± 80 ###	63 ± 39	892 ± 59 ####	21 ± 11

CON, control cells; ECHS1 KO, ECHS1 KO cells; UT, untreated; dNs, deoxyribonucleosides. Spare respiratory capacity equals the maximal rate-basal rate; the Cell respiratory control ratio equals the maximal rate divided by the proton leak rate.* p < 0.05, ** p < 0.01, *** p > 0.001, **** p > 0.0001 compared to untreated (ut) CON cells; # p < 0.05, ### p > 0.001, #### p > 0.0001 compared to untreated (ut) ECHS1 KO cells (Student’s two-tailed t tests).