Identification of Pristanal Dehydrogenase Activity in Peroxisomes: Conclusive Evidence That the Complete Phytanic Acid α-Oxidation Pathway Is Localized in Peroxisomes

Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolism

“…However, FALDH-V seems to be a membrane bound enzyme, whereas the peroxisomal pristanal dehydrogenation is catalyzed by a matrix enzyme. Hence, the link with SLS remains debatable: phytanic acid is not accumulating in this disorder ( 45 ) and ␣ -oxidation in SLS fi broblasts is normal ( 47 ). Pristanal likely can be converted by more than one peroxisomal enzyme and/or can leave the peroxisomal compartment and reenter as pristanic acid after oxidation at another site.…”

Peroxisomes and peroxisomal disorders: The main facts

“…Subsequently, after activation and transport to peroxisomes, the chain shortening of the methyl-branched side chain of THCA and DHCA is made by ȕ-oxidation (Wanders et al, 2001). …”

“…The biochemical anomalies correlated to these diseases present an accumulation of VLCFAs (C24:0, C25:0, C26:0), THCA and DHCA (from bile acids synthesis), branched-chain fatty acid (pristanic and phytanic acid) and a decrease in synthesis of plasmalogens and DHA (Jansen et al, 2001) 5.2 Peroxisomal enzyme/transporter deficiencies (PEDs)…”

Molecular Mechanisms and Physiological Significance of Organelle Interactions and Cooperation

“…This aldehyde is then converted into the corresponding acid (pristanic acid). Available evidence holds that peroxisomes do contain aldehyde dehydrogenase activity as shown for pristanal by Jansen et al (2001). The true identity of this enzyme activity has not been settled definitively.…”

“…The bulk of FALDH-activity produced from the ALDH3A2 gene, however, is located in the endoplasmic reticulum (ER). Whether FALDH-V is truly the enzyme responsible for the pristanal dehydrogenase activity in peroxisomes remains doubtful, however, for various reasons including the fact that FALDH-V appears to be a membrane-bound enzyme with its catalytic domain exposed to the cytosol, whereas the peroxisomal pristanal dehydrogenase activity is catalyzed by a soluble matrix enzyme (Jansen et al, 2001). Furthermore, phytanic acid alpha oxidation is fully normal in patients suffering from Sjögren Larsson syndrome (SLS) caused by mutations in the ALDH3A2 gene.…”