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phytanoyl-CoA dioxygenase family protein
This family is made up of several eukaryotic phytanoyl-CoA dioxygenase (PhyH) proteins, ectoine hydroxylases and a number of bacterial deoxygenases. PhyH is a peroxisomal enzyme catalysing the first step of phytanic acid alpha-oxidation. PhyH deficiency causes Refsum's disease (RD) which is an inherited neurological syndrome biochemically characterised by the accumulation of phytanic acid in plasma and tissues [1]. [1]. 10767344. Human phytanoyl-CoA hydroxylase: resolution of the gene structure and the molecular basis of Refsum's disease. Jansen GA, Hogenhout EM, Ferdinandusse S, Waterham HR, Ofman R, Jakobs C, Skjeldal OH, Wanders RJ;. Hum Mol Genet 2000;9:1195-1200. [2]. 15128576. Functional expression of the ectoine hydroxylase gene (thpD) from Streptomyces chrysomallus in Halomonas elongata. Prabhu J, Schauwecker F, Grammel N, Keller U, Bernhard M;. Appl Environ Microbiol. 2004;70:3130-3132. (from Pfam)
phytanoyl-CoA dioxygenase (PhyH) family protein may act as a dioxygenase that catalyzes the oxidation of a substrate without the reduction of one oxygen atom from dioxygen into a water molecule
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