The structure of the alpha-oxidation pathway and its subcellular localization has remained an enigma although there is convincing evidence that 2-hydroxyphytanic acid is an obligatory intermediate. We have now studied the degradation of 2-hydroxyphytanic acid in both rat and human liver. The results...
A previously unreported metabolite of the mammalian phytanic acid breakdown pathway, 2-oxophytanic acid, was isolated and analysed by mass spectrometry. The metabolic origin of the 2-oxoacid is the oxidation by a rat kidney peroxisomal H 2 O 2 -generating oxidase of L -2-hydroxyphytanic acid,...
Draye JP, van Hoof F, de Hoffmann E, Vamecq J 1987 Peroxisomal oxidation of L-2-hydroxyphytanic acid in rat kidney cortex. Eur J Biochem 167:573- 578Draye J., van Hoof F., de Hoffman E., Vamecq J. (1987) Peroxisomal oxidation of L-2- hydroxyphytanic acid in rat kidney cortex...
(1999) Phytanic acid α-oxidation: identification of 2-hydroxyphytanoyl-CoA lyase in rat liver and its localisation in peroxisomes. Biochim. Biophys. Acta, 1440, 176-182.Jansen GA, Verhoeven NM, Denis S, et al. Phytanic acid α-oxidation: identification of 2-hydroxyphytanoyl-CoA lyase in ...
Phytanic acid oxidation in man: identification of a new enzyme catalysing the formation of 2-ketophytanic acid from 2-hydroxyphytanic acid and its deficiency in the Zellweger syndrome. J. Inherit. Metab. Dis. 18, 201-203 (1995).Wanders, R. J. A., C. W. T. Van Roermund, D. S. M...
P. Draye: The enzymatic and mass spectrometric identification of 2-oxophytanic acid, a product of the peroxisomal oxidation of L-2-hydroxyphytanic acid. Biomed. Environ. Mass Spec- trom. 15, 345-351 (1988).Vaimecq, J, Draye, JP (1988) Biomed. Environ. Mass Spectrom. 15: pp. 345-...