Activation of peroxisome proliferator-activated receptor pathway stimulates the mitochondrial respiratory chain and can correct deficiencies in patients' cells lacking its components.

@article{Bastin2008ActivationOP,
  title={Activation of peroxisome proliferator-activated receptor pathway stimulates the mitochondrial respiratory chain and can correct deficiencies in patients' cells lacking its components.},
  author={Jean Bastin and Flore Aubey and Agn{\`e}s R{\"o}tig and Arnold Munnich and Fatima Djouadi},
  journal={The Journal of clinical endocrinology and metabolism},
  year={2008},
  volume={93 4},
  pages={1433-41}
}
CONTEXT The mitochondrial respiratory chain (RC) disorders are the largest group of inborn errors of metabolism and still remain without treatment in most cases. OBJECTIVE We tested whether bezafibrate, a drug acting as a peroxisome proliferator-activated receptor (PPAR) agonist, could stimulate RC capacities. DESIGN Fibroblasts or myoblasts from controls or patients deficient in complex I (CI), complex III (CIII), or complex IV (CIV) were cultured with or without bezafibrate. MAIN… CONTINUE READING