{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T08:16:39Z","timestamp":1790928999044,"version":"4.1.0"},"reference-count":36,"publisher":"Portland Press Ltd.","issue":"5","content-domain":{"domain":["portlandpress.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2007,11,1]]},"abstract":"<jats:p>In the present paper, we describe the current state of knowledge regarding the enzymology of the phytanic acid \u03b1-oxidation pathway. The product of phytanic acid \u03b1-oxidation, i.e. pristanic acid, undergoes three cycles of \u03b2-oxidation in peroxisomes after which the products, including 4,8-dimethylnonanoyl-CoA, propionyl-CoA and acetyl-CoA, are exported from the peroxisome via one of two routes, including (i) the carnitine-dependent route, mediated by CRAT (carnitine acetyltransferase) and CROT (carnitine O-octanoyltransferase), and (ii) the free acid route, mediated by one or more of the peroxisomal ACOTs (acyl-CoA thioesterases). We also describe our recent data on the \u03c9-oxidation of phytanic acid, especially since pharmacological up-regulation of this pathway may form the basis of a new treatment strategy for ARD (adult Refsum's disease). In patients suffering from ARD, phytanic acid accumulates in tissues and body fluids due to a defect in the \u03b1-oxidation system.<\/jats:p>","DOI":"10.1042\/bst0350865","type":"journal-article","created":{"date-parts":[[2007,11,1]],"date-time":"2007-11-01T11:09:43Z","timestamp":1193915383000},"page":"865-869","update-policy":"https:\/\/doi.org\/10.1042\/crossmark_policy","source":"Crossref","is-referenced-by-count":54,"title":["Peroxisomes, Refsum's disease and the \u03b1- and \u03c9-oxidation of phytanic acid"],"prefix":"10.1042","volume":"35","author":[{"given":"R.J.A.","family":"Wanders","sequence":"first","affiliation":[{"name":"Genetic Metabolic Diseases Laboratory, Room F0-224, Departments of Clinical Chemistry and Pediatrics, Academic Medical Center, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.C.","family":"Komen","sequence":"additional","affiliation":[{"name":"Genetic Metabolic Diseases Laboratory, Room F0-224, Departments of Clinical Chemistry and Pediatrics, Academic Medical Center, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"288","published-online":{"date-parts":[[2007,10,25]]},"reference":[{"key":"2021111900172573100_B1","doi-asserted-by":"crossref","first-page":"727","DOI":"10.1046\/j.0022-3042.2002.00766.x","volume":"80","author":"Wierzbicki","year":"2002","journal-title":"J. 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