Inhibition of 3-methylcrotonyl-CoA carboxylase explains the increased excretion of 3-hydroxyisovaleric acid in valproate-treated patients

@article{Lus2011InhibitionO3,
  title={Inhibition of 3-methylcrotonyl-CoA carboxylase explains the increased excretion of 3-hydroxyisovaleric acid in valproate-treated patients},
  author={Paula B. M. Lu{\'i}s and Jos P. N. Ruiter and Lodewijk van Ijlst and Lu{\'i}sa M Diogo and Paula Garc{\'i}a and Isabel Tavares de Almeida and M. X. Pe{\~n}a Dur{\'a}n and Ronald J. A. Wanders and Margarida F. B. Silva},
  journal={Journal of Inherited Metabolic Disease},
  year={2011},
  volume={35},
  pages={443-449}
}
Valproic acid (VPA) is a widely used anticonvulsant drug which affects mitochondrial metabolism including the catabolism of fatty acids and branched-chain amino acids. To elucidate the effect of valproate on the leucine pathway through a targeted metabolomics approach and the evaluation of the effects of valproate on the activity of biotinidase and 3… CONTINUE READING