Article
Metabolism of phytanic acid and 3-methyl-adipic acid excretion in patients with adult Refsum disease.
Journal of lipid research - 1 Aug 2003
Wierzbicki Anthony S, Mayne Phillip D, Lloyd Matthew D, Burston David, Mei Guam, Sidey Margaret C, Feher Michael D, Gibberd F Brian
Abstract excerpt
Adult Refsum disease (ARD) is associated with defective alpha-oxidation of phytanic acid (PA). omega-Oxidation of PA to 3-methyl-adipic acid (3-MAA) occurs although its clinical significance is unclear. In a 40 day study of a new ARD patient, where the plasma half-life of PA was 22.4 days, omega-...
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