title: Fatty acid omega-oxidation as a rescue pathway for fatty acid oxidation disorders in humans.
abstract: Fatty acids (FAs) can be degraded via different mechanisms including α-, β- and ω-oxidation. In humans, a range of different genetic diseases has been identified in which either mitochondrial FA β-oxidation, peroxisomal FA β-oxidation or FA α-oxidation is impaired. Treatment options for most of these disorders are limited. This has prompted us to study FA ω-oxidation as a rescue pathway for these disorders, based on the notion that if the ω-oxidation of specific FAs could be upregulated one could reduce the accumulation of these FAs and the subsequent detrimental effects in the different groups of disorders. In this minireview, we describe our current state of knowledge in this area with special emphasis on Refsum disease and X-linked adrenoleukodystrophy.
authors.affiliation: Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands. r.j.wanders@amc.uva.nl
authors.forename: Ronald J A
authors.initials: RJ
authors.lastname: Wanders
authors.forename: Jasper
authors.initials: J
authors.lastname: Komen
authors.forename: Stephan
authors.initials: S
authors.lastname: Kemp
mesh_terms: D000818:Animals; D003577:Cytochrome P-450 Enzyme System / Q000235:genetics / Q000378:metabolism; D004790:Enzyme Induction / Q000187:drug effects / Q000502:physiology; D005227:Fatty Acids / Q000378:metabolism*; D006801:Humans; D008052:Lipid Metabolism, Inborn Errors / Q000188:drug therapy* / Q000235:genetics / Q000378:metabolism*; D010084:Oxidation-Reduction
chemical_list: D005227:Fatty Acids; D003577:Cytochrome P-450 Enzyme System