Bezafibrate can be a new treatment option for mitochondrial fatty acid oxidation disorders: evaluation by in vitro probe acylcarnitine assay.

@article{Yamaguchi2012BezafibrateCB,
  title={Bezafibrate can be a new treatment option for mitochondrial fatty acid oxidation disorders: evaluation by in vitro probe acylcarnitine assay.},
  author={Seiji Yamaguchi and Hong Li and Jamiyan Purevsuren and Kenji Yamada and Midori Furui and Tomoo Takahashi and Yuichi Mushimoto and Hironori Kobayashi and Yuki Hasegawa and Takeshi Taketani and Toshiyuki Fukao and Seiji Fukuda},
  journal={Molecular genetics and metabolism},
  year={2012},
  volume={107 1-2},
  pages={87-91}
}
BACKGROUND The number of patients with mitochondrial fatty acid oxidation (FAO) disorders is recently becoming larger with the spread of newborn mass screening. Despite the advances in metabolic and molecular characterization of FAO disorders, the therapeutic studies are still limited. It was reported recently that bezafibrate (BEZ), an agonist of peroxisome proliferating activator receptor (PPAR), can restore FAO activity in cells from carnitine palmitoyltransferase-2 (CPT2) and very-long… CONTINUE READING

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