Fig. 1: Impact of fasting on metabolism in Pcca-/-(A138T) mice.

a Experimental design for fasting and a 20-min tracing study. b–e Changes in body weight, fat mass, fluid content, and lean mass observed in both fed and 23-h fasted Pcca-/-(A138T) mice. f Plasma levels of 3-hydroxybutyrate (BHB). g Schematic representation of C2 (acetylcarnitine) and C3 (propionylcarnitine) metabolism from C2-CoA (acetyl-CoA) and C3-CoA (propionyl-CoA). CrAT: carnitine acetyltransferase. h The ratio of propionylcarnitine (C3) to acetylcarnitine (C2) in plasma. i Illustration of the competitive interaction between C2-CoA (acetyl-CoA) and C3-CoA (propionyl-CoA) in the reaction with oxaloacetate (OAA) to produce citrate (Cit) and methylcitrate (Me-Cit). CS: citrate synthetase, TCAC: tricarboxylic acid cycle. j, k Plasma levels of methylcitrate and methylcitrate to citrate ratio. N = 5 per group. The error bar represents the SE. *, **, ***, and **** denote p values less than 0.05, 0.01, 0.005, and 0.001, respectively.