FIGURE 8.

CKF2 mice have decreased energy state in skeletal muscle. A, ATP levels in quadriceps muscle from WT and CKF2 mice were quantified by HPLC. Data are represented as mean ± S.E.; n = 5 mice per genotype. B, total AMPK, phosphorylated AMPK (p-AMPK), and total (ACC) and phosphorylated ACC (p-ACC) levels were determined by Western blot analysis following a 4-h fast in quadriceps muscle from WT and CKF2 mice. A representative blot and quantification are shown. Data are represented as mean ± S.E.; n = 4 mice per genotype. C, state 2 and state 3 mitochondrial ATP production rates were determined in mitochondria isolated from WT and CKF2 mice. *, p < 0.01; ***, p < 0.0001.