Fig. 6: Increased threonine metabolism in fasted Pcca-/-(A138T) mice.

a Schematic illustration of the metabolism of [13C4]threonine (M4 Threonine) to M3 propionylcarnitine (M3 C3 AC). SDS: Serine threonine dehydratase. M0, M3, and M4 denote the presence of 0, 3, and 4 heavy atoms, respectively, within a molecule. b–d Isotope labeling of M4 threonine, M4 2-hydroxybutyrate (2HB), and M3 propionylcarnitine (C3 AC), in plasma from both fed and fasted (23-h fasting) Pcca-/-(A138T) mice. e, f Levels of M0 C3 AC and M3 C3 AC in plasma from both fed and fasted (23-h fasting) Pcca-/-(A138T) mice. g Threonine dehydratase (SDS) activity in the liver of fed and fasted (23-h fasting) Pcca-/-(A138T) mice. N = 5 per group. The error bar represents the SE. *, **, ***, and **** indicate p values less than 0.05, 0.01, 0.005, and 0.001, respectively.