4508 Phytanic acid Phytanic acid (or 3,7,11,15-tetramethyl hexadecanoic acid) is a branched chain fatty acid that humans can obtain through the consumption of dairy products, ruminant animal fats, and certain fish. It is primarily formed by bacterial degradation of chlorophyll in the intestinal tract of ruminants. Unlike most fatty acids, phytanic acid cannot be metabolized by beta-oxidation (because of a methyl group in the beta position). Instead, it undergoes alpha-oxidation in the peroxisome, where it is converted into pristanic acid by the removal of one carbon. Pristanic acid can undergo several rounds of beta-oxidation in the peroxisome to form medium chain fatty acids that can be converted to carbon dioxide and water in mitochondria. Refsum disease, an autosomal recessive neurological disorder caused by mutations in the PHYH gene, is characterized by having impaired alpha-oxidation activity. Individuals with Refsum disease accumulate large stores of phytanic acid in their blood and tissues. This frequently leads to peripheral polyneuropathy, cerebellar ataxia, retinitis pigmentosa, anosmia, and hearing loss. Therefore, chronically high levels of phytanic acid can be neurotoxic. Phytanic acid’s neurotoxicity appears to lie in its ability to initiate astrocyte/neural cell death by activating the mitochondrial route of apoptosis. In particular, phytanic acid can induce substantial generation of reactive oxygen species in isolated mitochondria as well as in intact cells and it induces the release of cytochrome c from mitochondria. 14721-66-5 26840 C20H40O2 White powder. No indication of carcinogenicity to humans (not listed by IARC). This is an endogenously produced metabolite found in the human body. It is used in metabolic reactions, catabolic reactions or waste generation. 2014-08-29T06:51:24Z 2018-03-21T17:46:24Z Phytanic acid C01607 16285 true CC(C)CCCC(C)CCCC(C)CCCC(C)CC(O)=O C20H40O2 InChI=1/C20H40O2/c1-16(2)9-6-10-17(3)11-7-12-18(4)13-8-14-19(5)15-20(21)22/h16-19H,6-15H2,1-5H3,(H,21,22) InChIKey=RLCKHJSFHOZMDR-UHFFFAOYNA-N 312.5304 312.302830524 Endogenous Solid HMDB00801 25001 Karrer, P.; Epprecht, A.; Konig, Hans. General method of preparation for 2-methyl-3-alkylnaphthoquinones. Constitution and vitamin K activity. Helvetica Chimica Acta (1940), 23 272-83.