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Transcript
Proposed pathway for the oxidation of phytanic acid as suggested by Tsai et al.116 Based on studies in rat liver mitochondria, Tsai et al.116 concluded that
the first step in the degradation of phytanic acid is catalyzed by a mitochondrial NADPH- and O2-dependent ω-hydroxylase converting free phytanic acid to
free 2-hydroxyphytanic acid, followed by its decarboxylation to pristanic acid that, after activation to its CoA-ester, undergoes a series of successive βoxidations.
Source: Refsum Disease, The Online Metabolic and Molecular Bases of Inherited Disease
Citation: Valle D, Beaudet AL, Vogelstein B, Kinzler KW, Antonarakis SE, Ballabio A, Gibson K, Mitchell G. The Online Metabolic and Molecular
Bases of Inherited Disease; 2014 Available at: http://mhmedical.com/ Accessed: August 03, 2017
Copyright © 2017 McGraw-Hill Education. All rights reserved