ω-Hydroxylation of phytanic acid in rat liver microsomes
The 3-methyl-branched fatty acid phytanic acid is degraded by the peroxisomal α-oxidation route because the 3-methyl group blocks β-oxidation. In adult Refsum disease (ARD), peroxisomal α-oxidation is defective, which is caused by mutations in the gene coding for phytanoyl-CoA hydroxylase in the maj...
Main Authors: | , , |
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Format: | Article |
Language: | English |
Published: |
Elsevier
2004-07-01
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Series: | Journal of Lipid Research |
Subjects: | |
Online Access: | http://www.sciencedirect.com/science/article/pii/S0022227520317909 |