Walsh & Hoyt: Disorders of Peroxisomes

Peroxisomes are small subcellular organelles ranging in size from 0.2 to 1 micrometer. The organelles membrane is a single trilaminar structure. Peroxisomes are found in nearly every cell, except for mature erythyrocytes. Their distribution within individual tissues varies widely. They are most commonly found in the liver and kidney. Within the CNS, they are common in the oligodendrocytes, where they are typically located close to the developing myelin sheath and in neurons early in life. These organelles contain more than 40 enzymes. Characterization of these enzymes demonstrates that peroxisomes are involved in fatty acid oxidation, peroxide-forming cellular respiration, bile acid metabolism, and purine catabolism.