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lüll Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolism Van Veldhoven PPJ Lipid Res 2010[Oct]; 51 (10): 2863-95In humans, peroxisomes harbor a complex set of enzymes acting on various lipophilic carboxylic acids, organized in two basic pathways, alpha-oxidation and beta-oxidation; the latter pathway can also handle omega-oxidized compounds. Some oxidation products are crucial to human health (primary bile acids and polyunsaturated FAs), whereas other substrates have to be degraded in order to avoid neuropathology at a later age (very long-chain FAs and xenobiotic phytanic acid and pristanic acid). Whereas total absence of peroxisomes is lethal, single peroxisomal protein deficiencies can present with a mild or severe phenotype and are more informative to understand the pathogenic factors. The currently known single protein deficiencies equal about one-fourth of the number of proteins involved in peroxisomal FA metabolism. The biochemical properties of these proteins are highlighted, followed by an overview of the known diseases.|Animals[MESH]|Fatty Acids, Unsaturated/*metabolism[MESH]|Fatty Acids/*metabolism[MESH]|Humans[MESH]|Lipid Metabolism/physiology[MESH]|Oxidation-Reduction[MESH]|Peroxisomal Disorders/enzymology/*genetics[MESH]|Peroxisomes/*enzymology/metabolism[MESH]|Phytanic Acid/*metabolism[MESH] |