R

^-Oxidation

Figure 5 Peroxisomal phytanic acid a-oxidation pathway. The dietary 3-methyl-branched fatty acid phytanic acid is toxic if allowed to accumulate in the tissues. Its 3-methyl group prevents degradation by ^-oxidation; therefore, this fatty acid is first shortened by one carbon atom. Like the substrates for peroxisomal ^-oxidation, phytanic acid enters peroxisomes by an unknown mechanism. Activated phytanic acid is hydroxylated on carbon 2. Cleavage between carbons 1 and 2 yields a one-carbon CoA compound, formyl-CoA, and an aldehyde, pristanal. After oxidation and reactivation to the CoA derivative, pristanoyl-CoA can be degraded by ^-oxidation.

dicarboxylic acids can then be at least partially degraded by ^-oxidation from the !-end, primarily in peroxisomes.

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