Peroxisomal Fatty Acid bOxidation

Like mitochondria, peroxisomes contain pathways for the ^-oxidation of fatty acids. The mechanism by which fatty acids enter peroxisomes is unclear but does not appear to involve the CPT1-CACT-CPT2 pathway. Long-chain and very-long-chain acyl-CoA synthetase activities are associated with peroxisomes, but it has not been established whether fatty acids or fatty acyl-CoAs traverse the peroxisomal membrane. The basic reactions of per-oxisomal ^-oxidation resemble those found in mitochondria, but the peroxisomal and mitochondrial enzymes are distinct proteins (Figure 4). In fact, peroxisomes contain two sets of ^-oxidation enzymes, which appear to function with distinct substrates.

Unlike mitochondria, peroxisomes do not contain an electron transport chain or tricarboxylic acid cycle, and, thus, peroxisomal fatty-acid degradation is not directly coupled to energy production. Rather, peroxisomes have a more specialized fatty-acid oxidation role, degrading fatty-acid substrates that cannot be catabolized in mitochondria. Peroxisomes are indispensable for the degradation of very-long-chain fatty acids (containing more than 22 carbon atoms),

II II

Acetyl-CoA

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