Cardiac mitochondrial substrate selectivity was altered in the Taz KD mouse model. High-resolution respirometry demonstrated substrate-selective control of respiration utilizing (A) pyruvate, (B) palmitoyl-l-carnitine, (C) glutamate, and (D) pyruvate and glutamate substrates for state 3 respiration. E: Comparative analysis of substrate control ratios revealed an overall decrease in palmitoyl-l-carnitine-stimulated respiration and an increase in glutamate-stimulated respiration in Taz KD cardiac mitochondria compared with wild-type littermates. Values represent the mean oxygen consumption (nmol/min/mg mitochondrial protein) ± S.E. (N = 5 hearts per group; black bars, wild-type littermates; white bars, Taz KD mice). *P < 0.05 level, **P < 0.01 level.