Add time:08/07/2019         Source:sciencedirect.com

1.1. The metabolism of β-methyl fatty acids has been studied in healthy humans as well as in patients with Refsum's disease who show phytanic acid accumulation. As a test substance 3,6-dimethyl[8-14C]octanoic acid was used. This acid possesses a methyl group in the β-position to the carboxyl group, even after an initial ω-oxidation. The medium chain length facilitates the excretion of metabolites in the urine.2.2. During the first 7 h after the administration of 3,6-dimethyl[8-14C]octanoic acid about 65% of the radioactivity appeared in the urine. The main metabolites were: 7-hydroxy-3,6-dimethyloctanoic acid, 6-hydroxy-3,6-dimethyl-octanoic acid, 3,6-dimethyloctane-1,8-dioic acid, 7-keto-3,6-dimethyloctanoic acid, 3,6-dimethyl octanoic acid and 2,5-dimethylheptanoic acid.3.3. After ingestion of 3,6-dimethyl[8-14C]octanoic acid, healthy controls excreted 14CO2 in the expiratory air, and the metabolite 2,5-dimethylheptanoic acid was demonstrated in the urine. These findings show that 3,6-dimethyloctanoic acid can be degraded in man, and that an initial α-decarboxylation takes place, rendering the acid susceptible to normal β-oxidation.4.4. When 3,6-dimethyl [8-14C]octanoic acid was given to patients with Refsum's disease, no 14CO2 could be detected in the expiratory air, and no excretion of 2,5-dimethylheptanoic acid was found in the urine. The patients seem to lack the ability to degrade this acid.5.5. A failure to degrade β-methyl fatty acids by an α-decarboxylation apparently exists in patients with Refsum's disease. This failure may explain the accumulation of phytanic acid.

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