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4. Structure of human phytanoyl-CoA 2-hydroxylase identifies molecular mechanisms of Refsum disease. McDonough MA; Kavanagh KL; Butler D; Searls T; Oppermann U; Schofield CJ J Biol Chem; 2005 Dec; 280(49):41101-10. PubMed ID: 16186124 [TBL] [Abstract][Full Text] [Related]
5. Phytanoyl-CoA hydroxylase: recognition of 3-methyl-branched acyl-coAs and requirement for GTP or ATP and Mg(2+) in addition to its known hydroxylation cofactors. Croes K; Foulon V; Casteels M; Van Veldhoven PP; Mannaerts GP J Lipid Res; 2000 Apr; 41(4):629-36. PubMed ID: 10744784 [TBL] [Abstract][Full Text] [Related]
6. Studies on phytanoyl-CoA 2-hydroxylase and synthesis of phytanoyl-coenzyme A. Kershaw NJ; Mukherji M; MacKinnon CH; Claridge TD; Odell B; Wierzbicki AS; Lloyd MD; Schofield CJ Bioorg Med Chem Lett; 2001 Sep; 11(18):2545-8. PubMed ID: 11549466 [TBL] [Abstract][Full Text] [Related]
7. Restoration of phytanic acid oxidation in Refsum disease fibroblasts from patients with mutations in the phytanoyl-CoA hydroxylase gene. Chahal A; Khan M; Pai SG; Barbosa E; Singh I FEBS Lett; 1998 Jun; 429(1):119-22. PubMed ID: 9657395 [TBL] [Abstract][Full Text] [Related]
10. Phytanic acid oxidation: normal activation and transport yet defective alpha-hydroxylation of phytanic acid in peroxisomes from Refsum disease and rhizomelic chondrodysplasia punctata. Pahan K; Khan M; Singh I J Lipid Res; 1996 May; 37(5):1137-43. PubMed ID: 8725164 [TBL] [Abstract][Full Text] [Related]
11. Identification of a brain specific protein that associates with a refsum disease gene product, phytanoyl-CoA alpha-hydroxylase. Lee ZH; Kim H; Ahn KY; Seo KH; Kim JK; Bae CS; Kim KK Brain Res Mol Brain Res; 2000 Feb; 75(2):237-47. PubMed ID: 10686344 [TBL] [Abstract][Full Text] [Related]
12. Brain pyruvate and 2-oxoglutarate dehydrogenase complexes are mitochondrial targets of the CoA ester of the Refsum disease marker phytanic acid. Bunik VI; Raddatz G; Wanders RJ; Reiser G FEBS Lett; 2006 Jun; 580(14):3551-7. PubMed ID: 16737698 [TBL] [Abstract][Full Text] [Related]
13. Phytanic acid activation in rat liver peroxisomes is catalyzed by long-chain acyl-CoA synthetase. Watkins PA; Howard AE; Gould SJ; Avigan J; Mihalik SJ J Lipid Res; 1996 Nov; 37(11):2288-95. PubMed ID: 8978480 [TBL] [Abstract][Full Text] [Related]
14. Omega-hydroxylation of phytanic acid in rat liver microsomes: implications for Refsum disease. Komen JC; Duran M; Wanders RJ J Lipid Res; 2004 Jul; 45(7):1341-6. PubMed ID: 15102880 [TBL] [Abstract][Full Text] [Related]
16. Role of phytanoyl-CoA 2-hydroxylase in phytanic acid metabolism. Lloyd MD; Mukherji M; Kershaw NJ; Chien W; Wierzbicki AS; Schofield CJ Adv Exp Med Biol; 2003; 544():303-4. PubMed ID: 14713244 [No Abstract] [Full Text] [Related]
17. Phytanic acid must be activated to phytanoyl-CoA prior to its alpha-oxidation in rat liver peroxisomes. Watkins PA; Howard AE; Mihalik SJ Biochim Biophys Acta; 1994 Oct; 1214(3):288-94. PubMed ID: 7918611 [TBL] [Abstract][Full Text] [Related]
18. Molecular basis of Refsum disease: sequence variations in phytanoyl-CoA hydroxylase (PHYH) and the PTS2 receptor (PEX7). Jansen GA; Waterham HR; Wanders RJ Hum Mutat; 2004 Mar; 23(3):209-18. PubMed ID: 14974078 [TBL] [Abstract][Full Text] [Related]