Table 2:

Laboratory and MRS measurements of the patients and of the control subjects

Patients	Cr per Os (mg/kg/bw/day)	Plasmatic Cr (μmol/L)	Plasmatic GAA (μmol/L)	Brain Cr (mmol/L)	PCr/PDE	P_i/PDE	ATP/PDE	pH
GAMT-d
Patient 1	800	487	11.4	3.86	1.03	0.76	0.58	7.22
	400^*	598	11.1	3.10	0.91	0.75	0.59	6.85
	400^**	512	7.9	3.31	1.19	0.70	0.65	7.43
Patient 2	0	24	40.3	0.93	0.37^†	0.72	0.80	7.08
	400^**	431	7.4	3.76	1.02	0.78	0.57	6.75
AGAT-d
Patient 3	0	6		1.10	0.73	0.84	0.63	7.31
	400	776		3.87	1.24	0.85	0.61	7.21
	200	203		3.65	1.19	0.82	0.70	7.20
Patient 4	0	6		1.05	0.76	0.87	0.64	7.31
	400	588		4.00	1.49	0.90	0.65	7.17
	200	178		3.85	1.15	0.81	0.77	7.36
Patient 5	0	8		1.33	0.76	0.87	0.64	7.22
	400	283		3.99	1.32	0.87	0.64	7.04
	200	93		3.52	0.83	0.76	0.62	7.54
	300	246		3.61	0.93	0.77	0.54	7.22
Reference values	n.v. ± SD	64 ± 14	1.48 ± 0.51	4.37 ± 0.44	1.40 ± 0.14	0.87 ± 0.08	0.64 ± 0.06	7.15 ± 0.12

Note:—Os indicates oral intake; Cr, creatine; bw, body weight; GAA, guanidinoacetate; PCr, phosphocreatine; PDE, phosphodiester; P_i, inorganic phosphate; GAMT-d indicates guanidinoacetate methyltransferase defect; AGAT-d, arginine:glycine amidinotransferase defect; n.v., normal value.
* Coupled with ornithine supplementation.
** Coupled with ornithine supplementation and arginine restriction.
† Resonance frequency of this signal was at −0.5 ppm and was assigned to GAA-P.