Definitions

from Wiktionary, Creative Commons Attribution/Share-Alike License

  • n. A univalent radical formally derived from phytanic acid by removal of the hydroxyl group

Etymologies

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Examples

  • Hogenhout EM, Ferdinandusse S, Waterham HR, Ofman R, et al. (2000) Human phytanoyl-CoA hydroxylase: resolution of the gene structure and the molecular basis of Refsum's disease.

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  • Ofman R, Ferdinandusse S, Ijlst L, Muijsers AO, et al. (1997) Refsum disease is caused by mutations in the phytanoyl-CoA hydroxylase gene.

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  • Characterization of phytanoyl-Coenzyme A hydroxylase in human liver and activity measurements in patients with peroxisomal disorders.

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  • Mus musculus (mouse [9]) [9] 3 Reaction and Specificity Catalyzed reaction phytanoyl-CoA + 2-oxoglutarate + O2 = 2-hydroxyphytanoyl-CoA + succi - nate + CO2 Reaction type oxidation redox reaction reduction 111

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  • A.; Khan, M.; Pai, S.G.; Barbosa, E.; Singh, I.: Restoration of phyta - nic acid oxidation in Refsum disease fibroblasts from patients with muta - tions in the phytanoyl-CoA hydroxylase gene.

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  • Watkins PA, Howard AE, Mihalik SJ (1994) Phytanic acid must be activated to phytanoyl-CoA prior to its alpha-oxidation in rat liver peroxisomes.

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  • Refsum disease is caused by a deficiency of phytanoyl-CoA hydroxylase (PHYH), the first enzyme of the peroxisomal α-oxidation system, resulting in the accumulation of the branched-chain fatty acid phytanic acid.

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  • < 1, 2 > [10]) [10] P 2-hydroxy-3-methylhexadecanoyl-CoA + succinate + CO2 S phytanoyl-CoA + 2-oxoglutarate + O2

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