Human Bifunctional Enzyme and Its Import into Peroxisomes
Peroxisomes are present in almost all mammalian cells other than mature erythrocytes, but their size and number vary considerably. In the nervous system they are more abundant during development, specifically in the period of active myelination, and the early postnatal period. Peroxisomes contain more than 40 enzymes and play an important role in intermediary metabolism. In addition to the production of H2O2 (by oxidase) and its degradation (by catalase), peroxisomes are involved in a large variety of catabolic (oxidation of fatty acids, ethanol, Lpipecolic acid, polyamine and purines), and anabolic reactions (biosynthesis of bile acids, cholesterol and ether-linked phospholipids, such as plasmalogens, an essential myelin constituent) (Lazarow and Moser, 1989).
KeywordsBile Acid Bifunctional Enzyme Pipecolic Acid Zellweger Syndrome Peroxisomal Target Signal
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