Alpha-oxidation of Fatty Acids in Various Mammals, and a Phytanic Acid Feeding Experiment in an Animal with a Low Alpha-oxidation Capacity

Abstract

Patients with Refsum's disease, who accumulate phytanic acid, have a reduced capacity for α-oxidation of branched chain fatty acids. To try to elucidate the role of phytanic acid in the symptomatology of this disease, a phytanic acid feeding experiment in animals has been performed. In vitro studies with 3,6-dimethyloctanoic acid showed that carnivorous animals had lower α-oxidation capacities than herbivorous animals. Polecats (Mustela putorius) were chosen for the phytanic acid feeding, which was started in utero, before the main myelination had occurred, and continued until they had reached maturity weights.During the feeding period the animals increased their tolerance for phytanic acid by increasing their α-oxidation capacity. Large amounts of phytanic acid had acute toxic effects, but no symptoms of myelin disorders occurred during the experiment. Based upon these findings, compared with the clinical improvements of the patients on a phytanic acid lowering diet, the pathogenic role of phytanic acid is discussed.