acids, D-amino acids, urate, xanthine and very-long-chain fatty acids) cells. Mitochondria are the site of the citric acid (Krebs’) cycle and the
and produce hydrogen peroxide that is detoxified (degraded) by per- electron transport (cytochrome) pathway by which complex organic
oxisomal catalase. Peroxisomes are particularly numerous in hepato- molecules are finally oxidized to carbon dioxide and water. This process
cytes. Peroxisomes are important in the oxidative detoxification of provides the energy to drive the production of ATP from adenosine
various substances taken into or produced within cells, including diphosphate (ADP) and inorganic phosphate (oxidative phosphoryla-
ethanol. Peroxin mutation is a characteristic feature of Zellweger syn- tion). The various enzymes of the citric acid cycle are located in the
drome (craniofacial dysmorphism and malformations of brain, liver, mitochondrial matrix, whereas those of the cytochrome system and
eye and kidney; cerebrohepatorenal syndrome). Neonatal leukodystro- oxidative phosphorylation are localized chiefly in the inner mitochon-