Table 2.

Plasma concentrations of all amino acids (µmol/l) in the two patients

Amino acid	Patient 1	Patient 2	Reference values
Taurine	21	72	9–130
Threonine	61	29	49–183
Serine	31	33	95–166
Asparagine	18	6	18–69
Glutamic acid	23	28	1–105
Glutamine	301	262	317–757
Proline	70	149	26–412
Glycine	107	185	139–317
Alanine	72	181	153–560
Citrulline	15	4	10–57
Valine	158	73	120–323
Cystine	6	18	0–42
Methionine	9	7	13–38
Isoleucine	59	13	24–100
Leucine	120	48	49–179
Tyrosine	36	43	30–109
Phenylalanine	70	46	28–92
Ornithine	34	12	30–96
Lysine	96	47	81–217
Histidine	89	47	55–104
Arginine	38	31	29–124