Different ratios of the ldd and ddd diastereoisomers of phytanic acid in patients with Refsum's disease

Abstract

1. 1. Both the ldd and ddd diastereoisomers of phytanic acid accumulate in patients with Refsum's disease.Considerable differences occur in the ldd/ ddd ratios of the phytanic acid in these patients, probably depending on differences in the diet. 2. 2. The limited degradation of phytanic acid which takes place in patients with Refsum's disease removes the ldd and ddd isomers at approximately the same rate. 3. 3. Experiments with polecats also indicate that the rapid degradation of phytanic acid which takes place in normal mammals removes the ldd and ddd isomers at approximately the same rate. 4. 4. The distribution of the ldd and ddd isomers of phytanic acid among the various lipid fractions in serum indicates that the ldd and ddd isomers have different kinetics for several acyl activating systems and/or acyltransferases. 5. 5. Administration of phytol to the polecat gives rise to an accumulation of phytanic acid with a preponderance of the ldd isomer.