Phytanic acid alpha-oxidation: decarboxylation of 2-hydroxyphytanoyl-CoA to pristanic acid in human liver.

@article{Verhoeven1997PhytanicAA,
  title={Phytanic acid alpha-oxidation: decarboxylation of 2-hydroxyphytanoyl-CoA to pristanic acid in human liver.},
  author={Nanda M. Verhoeven and Ronald J. A. Wanders and Danielle S M Schor and Gerbert A. Jansen and Cornelis Jakobs},
  journal={Journal of lipid research},
  year={1997},
  volume={38 10},
  pages={2062-70}
}
The degradation of the first intermediate in the alpha-oxidation of phytanic acid, 2-hydroxyphytanoyl-CoA, was investigated. Human liver homogenates were incubated with 2-hydroxyphytanoyl-CoA or 2-hydroxyphytanic acid, after which formation of 2-ketophytanic acid and pristanic acid were studied. 2-Hydroxyphytanic acid was converted into 2-ketophytanic acid and pristanic acid. When ATP, Mg2+, and coenzyme A were added to the incubation medium, higher amounts of pristanic acid were formed… CONTINUE READING