Tazkd results in altered CL levels and protects from weight gain. (A) Tafazzin mRNA levels in the heart and (B) in the skeletal muscle of wild‐type and Tazkd mice. (C) Upper, western blot of tafazzin protein in heart extracts of 3‐ and 12‐month‐old wild‐type and tazkd mice and in skeletal muscle extracts of 12‐month‐old wild‐type and Tazkd mice probed with anti‐tafazzin antibodies [36]. VDAC was used as a loading control. Bottom, densitometric analysis of western blots from the upper panel C, tafazzin levels were normalized by VDAC levels, NS, nonspecific band. (D) MLCL and CL levels were determined by high‐performance liquid chromatography in heart and skeletal muscle isolated mitochondria from wild‐type and tazkd mice; an elevated ratio of MLC L/CL indicates a defect in CL remodeling. (E) Body weights of wild‐type and tazkd mice. (F) Heart weight normalized by total body weight. Values are mean ± SEM,n ≧ 3. *P < 0.05; ***P < 0.001; ****P< 0.0001, using Student's t‐test. **, 2‐way ANOVA, Sidak's post‐test.