Fig. 7: Enhanced gluconeogenesis increases the disposal of propionyl-CoA in fasted Pcca-/-(A138T) mice.

a Schematic representation of the catabolism of [13C3]propionate to the tricarboxylic acid (TCA) cycle and gluconeogenesis. OAA: oxaloacetate, PEP: phosphoenolpyruvate. b–d Stable isotope labeling of M3 propionylcarnitine (M3 C3 AC), average carbon labeling of citrate (Citrate avg C labeling), and average carbon labeling of glucose (Glucose avg C labeling) from [13C3]propionate in the plasma of both fed and fasted (23-h fasting) Pcca-/-(A138T) mice. e Changes in citrate levels in the liver, heart, brain, Quad, WAT, and kidney after a 23-h fasting. M3 denotes the presence of 3 heavy atoms within a molecule. Quad: quadriceps; WAT: white adipose tissue. 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.