{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T20:52:42Z","timestamp":1780606362586,"version":"3.54.1"},"reference-count":29,"publisher":"Wiley","issue":"13","license":[{"start":{"date-parts":[[2001,12,25]],"date-time":"2001-12-25T00:00:00Z","timestamp":1009238400000},"content-version":"vor","delay-in-days":542,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["European Journal of Biochemistry"],"published-print":{"date-parts":[[2000,7]]},"abstract":"<jats:p>Phytanic acid (3,7,11,15\u2010tetramethylhexadecanoic acid) is a branched\u2010chain fatty acid present in various dietary products such as milk, cheese and fish. In patients with Refsum disease, accumulation of phytanic acid occurs due to a deficiency of phytanoyl\u2010CoA hydroxylase, a peroxisomal enzyme containing a peroxisomal targeting signal 2. Recently, phytanoyl\u2010CoA hydroxylase cDNA has been isolated and functional mutations have been identified. As it has been shown that phytanic acid activates the nuclear hormone receptors peroxisome proliferator\u2010activated receptor (PPAR)\u03b1 and all three retinoid X receptors (RXRs), the intracellular concentration of this fatty acid should be tightly regulated.<\/jats:p><jats:p>When various cell lines were grown in the presence of phytanic acid, the activity of phytanoyl\u2010CoA hydroxylase increased up to four times, depending on the particular cell type. In one cell line, HepG2, no induction of phytanoyl\u2010CoA hydroxylase activity was observed. After addition of phytanic acid to COS\u20101 cells, an increase in phytanoyl\u2010CoA hydroxylase activity was observed within 2\u2003h, indicating a quick cell response. No stimulation of phytanoyl\u2010CoA hydroxylase was observed when COS\u20101 cells were grown in the presence of clofibric acid, 9\u2010<jats:italic>cis<\/jats:italic>\u2010retinoic acid or both ligands together. This indicates that the activation of phytanoyl\u2010CoA hydroxylase is not regulated via PPAR\u03b1 or RXR. However, stimulation of PPAR\u03b1 and all RXRs by clofibric acid and 9\u2010<jats:italic>cis<\/jats:italic>\u2010retinoic acid was observed in transient transfection assays. These results suggest that the induction of phytanoyl\u2010CoA hydroxylase by phytanic acid does not proceed via one of the nuclear hormone receptors, RXR or PPAR\u03b1.<\/jats:p>","DOI":"10.1046\/j.1432-1327.2000.01451.x","type":"journal-article","created":{"date-parts":[[2003,3,11]],"date-time":"2003-03-11T17:47:33Z","timestamp":1047404853000},"page":"4063-4067","source":"Crossref","is-referenced-by-count":19,"title":["Phytanoyl\u2010CoA hydroxylase activity is induced by phytanic acid"],"prefix":"10.1111","volume":"267","author":[{"given":"Anna W. 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