Table 1. Comparison of metabolite levels in urine and blood of the mouse models.^* .

Mouse genotype		Urine methylmalonic acid (µmol/mmol creatinine)	C3 propionylcarnitine (µmol/L)
Control adult ( Mut +/+)		119±83 (48)	0.8±0.2 (31)
MMA stop codon adult ( Mut +/+ MUT Stop+/−)		115±70 (22)	0.8±0.2 (18)
MMA stop codon rescue adult ( Mut−/−MUT Stop+/− MUT2h +/− )		23400±7800 (25)	12.8±4.3 (9)
Control pup ( Mut +/+)	1 day prior to birth	N/A	2.0±0.5 (15)
	At birth	123±22 (6)	1.9±0.3 (9)
	At 16 hours	109±11 (8)	2.2±0.5 (20)
MMA Knockout pup ( Mut−/− )	1 day prior to birth	N/A	10.0±2.5 (4)
	At birth	5710±2293 (5)	14.1±1.7 (3)
	At 16 hours	20054±6746 (5)	16.9±4.7 (10)
MMA Knockout stop codon pup ( Mut−/−MUT Stop+/−)	1 day prior to birth	N/A	11.2±3.5 (6)
	At birth	3208±1045 (3)	14.7±3.0 (3)
	At 16 hours	N/A	17.7±3.0 (3)
MMA stop codon rescue pup ( Mut−/−MUT Stop+/− MUT2h +/− )	1 day prior to birth	N/A	2.3±0.3 (2)
	At birth	1406±94 (2)	4.5±1.0 (2)
	At 16 hours	2679±910 (4)	5.0±1.5 (4)

^*Adult mice at 6 weeks old. Results presented as mean ± SEM. Number of samples in parenthesis.