Figure 1.

Mitochondrial biogenesis in FLCN-deficient muscle. A) Mice carrying FLCN alleles flanked by loxP sites (floxed, f) were crossed with CKM (muscle creatine kinase)– Cre transgenic mice to generate muscle-specific FLCN knockout mice (FLCN ^f/f /CKM– Cre). Pictures show representative images of muscle with indicated genotypes. B) Mitochondrial gene mRNA levels in quadriceps muscle tissue from littermate-matched mice were quantified with the indicated genes by real-time polymerase chain reaction. * Cytochrome C. Mean±95% confidence interval (CI). n=6. Student’s t test (two-sided). C) Lysates of quadriceps muscle from littermate-matched mice were immunoblotted with the indicated antibodies. *Cytochrome C. β-actin served as a loading control. D) Electron microscopy images were obtained from gastrocnemius muscle tissues of littermate matched mice. ×1000 (scale bar: 2 µm) and ×15 000 (scale bar: 500nm) magnified images. Arrows indicate mitochondria. Right bars represent percentage of mitochondrial area. Mean ± 95% confidence interval. Student’s t test (two-sided).