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. 2013 Jun;4(3):101–114. doi: 10.1177/2042098613479393

Figure 3.

Figure 3.

Schematic diagram showing metabolic changes upon dosing with compound A. Phenylacetylglycine (PAG) is formed metabolically from phenylalanine (PA) via phenylacetyl coenzyme A (CoA) and phenylpyruvate. The metabolism of PA is linked to the mitochondrial tricarboxylic acid (TCA) cycle via acetyl CoA. Dosing with compound A but not compound B caused an increase in urinary PAG (as indicated schematically by yellow arrows), and the higher dose of compound A caused disruption to the TCA cycle as indicated by a decrease in TCA cycle intermediates compared with the control. Downregulation of phenylalanine hydroxylase (PAH) by compound A after 48 h correlated with a decrease in tyrosine levels. An increase in urinary inorganic phosphate also indicated disruption to oxidative phosphorylation with compound A. (Adapted from the Krebs cycle and Kaufman [1999].) AA, amino acid; ADP, adenosine diphosphate; ATP, adenosine triphosphate; CPT1, carnitine palmitoyltransferase 1; DOPA, dihydroxyphenylalanine; FADH2, reduced form of flavin adenine dinucleotide; GTP, guanine triphosphate; NADH, nicotinamide adenine dinucleotide plus hydrogen.