@inbook{43e556d1a5104270a8c0aaafc80e578e,
title = "Role of phytanoyl-CoA 2-hydroxylase in phytanic acid metabolism",
abstract = "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{\textquoteright}s disease, in which phytanic acid accumulates (Wanders et al., 2001; Wierzbicki et al., 2002).",
author = "Lloyd, {Matthew D.} and Mridul Mukherji and Kershaw, {Nadia J.} and Winnie Chien and Wierzbicki, {Anthony S.} and Schofield, {Christopher J.}",
year = "2003",
month = dec,
day = "1",
doi = "10.1007/978-1-4419-9072-3_38",
language = "English",
isbn = "9781461347828",
volume = "544",
series = "Advances in Experimental Medicine and Biology",
publisher = "Springer Science + Business Media",
pages = "303--304",
editor = "Frank Roels and Myriam Baes and {De Bie}, Sylvia",
booktitle = "Peroxisomal disorders and regulation of genes",
address = "USA United States",
}