Abstract:
:Patients suffering from Refsum disease have a defect in the alpha-oxidation pathway which results in the accumulation of phytanic acid in plasma and tissues. Our previous studies have shown that phytanic acid is also a substrate for the omega-oxidation pathway. With the use of specific inhibitors we now show that members of the cytochrome P450 (CYP450) family 4 class are responsible for phytanic acid omega-hydroxylation. Incubations with microsomes containing human recombinant CYP450s (Supersomes) revealed that multiple CYP450 enzymes of the family 4 class are able to omega-hydroxylate phytanic acid with the following order of efficiency: CYP4F3A>CYP4F3B>CYP4F2>CYP4A11.
journal_name
FEBS Lettjournal_title
FEBS lettersauthors
Komen JC,Wanders RJdoi
10.1016/j.febslet.2006.05.069subject
Has Abstractpub_date
2006-07-10 00:00:00pages
3794-8issue
16eissn
0014-5793issn
1873-3468pii
S0014-5793(06)00686-7journal_volume
580pub_type
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