Phytanoyl-CoA 2-hydroxylase (PAHX), an iron(II) and 2-oxoglutarate-dependen
t oxygenase, catalyses an essential step in the mammalian metabolism of bet
a -methylated fatty acids. Phytanoyl-CoA was synthesised and used to develo
p in vitro assays for PAHX. The product of the reaction was confirmed as 2-
hydroxyphytanoyl-CoA by NMR and mass spectrometric analyses. In accord with
in vivo analyses, hydroxylation of both 3R and 3S epimers of the substrate
was catalysed by PAHX. Both pro- and mature- forms of PAHX were fully acti
ve. (C) 2001 Elsevier Science Ltd. All rights reserved.