Lipid metabolism in peroxisomes: enzymology, functions and dysfunctions of the fatty acid alpha- and beta-oxidation systems in humans.

摘要: Peroxisomes are subcellular organelles present in virtually all eukaryotic cells catalysing a number of indispensable functions cellular metabolism. The importance peroxisomes man is stressed by the existence an expanding group genetic diseases which there impairment one or more peroxisomal functions. One major concerns their role lipid metabolism, includes: (i) fatty acid betaoxidation; (ii) ether phospholipid synthesis; (iii) alpha-oxidation; and (iv) isoprenoid biosynthesis. In this paper, we review current state knowledge concerning alpha- beta-oxidation systems with particular emphasis on enzymes involved various disorders oxidation peroxisomes. We also pay attention to fact that some metabolites accumulate as result defect and/or activators members family nuclear receptors, including peroxisome-proliferator-activated receptor alpha.