Role of phytanoyl-CoA 2-hydroxylase in phytanic acid metabolism

Matthew D. Lloyd, Mridul Mukherji, Nadia J. Kershaw, Winnie Chien, Anthony S. Wierzbicki, Christopher J. Schofield

Research output: Chapter or section in a book/report/conference proceedingChapter or section

1 Citation (SciVal)


Phytanic acid is a 3-methyl branched fatty acid that cannot be degraded by P-oxidation. Instead, one carbon atom is removed to give pristanic acid by a single round of peroxisomal a-oxidation. Phytanoyl-CoA 2- hydroxylase (PAHX), an iron(II) and 2-oxoglutarate oxygenase, catalyses the first step in this pathway. Defects in PAHX cause some forms of adult Refsum’s disease, in which phytanic acid accumulates (Wanders et al., 2001; Wierzbicki et al., 2002).

Original languageEnglish
Title of host publicationPeroxisomal disorders and regulation of genes
EditorsFrank Roels, Myriam Baes, Sylvia De Bie
Place of PublicationNew York
PublisherSpringer Science + Business Media
Number of pages2
ISBN (Electronic)9781441990723
ISBN (Print)9781461347828
Publication statusPublished - 1 Dec 2003

Publication series

NameAdvances in Experimental Medicine and Biology
ISSN (Print)0065-2598

ASJC Scopus subject areas

  • General Biochemistry,Genetics and Molecular Biology


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