Supplemental Fig. 2

Concentrations of other amino acids, including non-protein amino acids and metabolites, in brain, liver, and sera derived from control and PKU mice receiving 18 %, 6 %, and 4 % protein diets for 3 weeks. Abbreviations employed: cit, citrulline; tau, taurine; PEA, phosphoethanolamine; AADA, 2-aminoadipic acid; AABA, α-aminobutyric acid. AADA represents a metabolite on the lysine/saccharopine catabolic pathway. AABA is also recognized structurally as homoalanine, and is generated via transamination of 2-oxobutyrate, a metabolite in the isoleucine biosynthetic pathway. Other parameters are described in the legend for Fig. 1. Neither AADA nor AABA were detectable in sera. (GIF 182 kb)