Figure 1. Metabolite Profile in Arginase Deficiency.

Z-scores for metabolites in subjects with arginase deficiency were averaged. All metabolite z-scores that were mapped to the pathway map are plotted. Only metabolites with z-scores ≥ 2 or ≤ - 2 were plotted. Blue nodes indicate negative z-scores, whereas red nodes indicate positive z-scores. Node size reflects the strength of the z-score. Numbers refer to particular pathways and biological processes, described below.

1–Glycosylation 2–Ascorbate Metabolism 3–Glycogen Metabolism

4–Galactose Metabolism 5–Inositol Metabolism 6–BCAA Metabolism

7–Riboflavin Metabolism 8–Pentose Phosphate Pathway 9–Pyrimidine Metabolism

10–Sorbitol/Glycerol Metabolism 11–Benzoate Metabolism 12–Purine Metabolism

13–Protein Degradation 14–Lysine Catabolism 15–Glycolysis

16–Glycine/Serine/Threonine 17–Carnitine Biosynthesis 18–Methionine/Cysteine

Metabolism Metabolism

19–Glutathione Metabolism 20–Histidine Metabolism 21–Ketone Bodies

22–Nicotinate/Nicotinamide 23–Mevalonate Metabolism 24–Tryptophan

Metabolism Metabolism

25–TCA Cycle 26–Arginine Metabolism and 27–Eicosanoids

Urea Cycle

28–Polyamine Metabolism 29–Creatine Metabolism 30–Aspartate/Glutamate

Metabolism

31–Phenylalanine/Tyrosine 32–Proline Metabolism 33–Endocannabinoid

Metabolism Synthesis

34–Fibrinogen Cleavage Peptides 35–Sphingolipid Metabolism 36–Phospholipid

Metabolism

37–Thyroid Hormone Synthesis 38–Fatty Acid Metabolism 39 Hemoglobin/Porphyrin

Metabolism

40–Steroid Hormone Biosynthesis 41–Bile Acid Metabolism 42–Dicarboxylic Acid

Metabolism

43-Secondary Bile Acids