Patterns of leucine-related liver acyl-CoAs and urine and plasma metabolites.

<p>The intermediates of leucine degradation are shown on the left. The metabolic block at HL is shown. Related urine and plasma metabolites are shown on the right. The number of arrowheads reflects the magnitude of increase with respect to normal, nonstressed control mice for HLLKO mice under normal conditions (unfilled arrows) and during decompensations filled arrows: 1 arrowhead, 3 to <9-fold increase; 2, 9-<27X; 3, 27-<81X; 4, >81X. Abbreviations, IVA-CoA, isovaleryl-CoA; 3-MC-CoA: 3-methylcrotonyl-CoA; 3-MGO-CoA, 3-methylglutaconyl-CoA; 3-OH-IVA-CoA, 3-hydroxyisovaleryl-CoA; C5OH-carn: C5 hydroxycarnitine; 3-OH-IVA, 3-hydroxyisovaleric acid; 3-MC-gly, 3-methylcrotonylglycine; 3-MGO, 3-methylglutaconic acid; 3-MG, 3-methylglutaric acid. NA, not available (see <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0060581#pone.0060581.s005" target="_blank">text</a>).