Figure 5. Ketogenic diet normalizes the CS-Mpc2−/− heart failure and glycogen accumulation.

A, Heart weight to tibia length ratio (HW/TL) of fl/fl and CS-Mpc2−/− mice fed either low fat (LF) or ketogenic diet (KD), n=4–7. B, Heart glycogen content measured biochemically in fl/fl and CS-Mpc2−/− mice fed either a LF diet or KD, n=4–7. C, Representative transmission electron microscopy images at 5,000X magnification of the cardiac papillary muscle of fl/fl and CS-Mpc2−/− mice fed LF or KD. Arrowheads denote glycogen granules. Scale bars = 1 μm. D, Western blot images of phosphorylated glycogen synthase (serine 640) and total glycogen synthase from hearts of fl/fl or CS-Mpc2−/− mice fed either LF or KD, n=2. E through J, ¹³C % enrichment of glucose (E) and the glucose within glycogen (F), and the normalized pool size of glucose-6-phosphate (G6P) (G), pyruvate (H), uridine diphosphate (UDP)-glucose (I), and glycogen (J) in fl/fl and CS-Mpc2−/− mice that were fed LF or KD and injected i.p. with U-¹³C-glucose, n=6–7. Data are presented as the mean±SD, and were analyzed by two-way analysis of variance (ANOVA) with Tukey post-hoc multiple-comparisons test.