Beta-oxidation of pristanoyl-CoA

Stable Identifier
R-HSA-389887
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Pathway
Species
Homo sapiens
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Pristanoyl-CoA, generated in the peroxisome by alpha-oxidation of dietary phytanic acid, is further catabolized by three cycles of peroxisomal beta-oxidation to yield 4,8-dimethylnonanoyl-CoA, acetyl-CoA and two molecules of propionyl-CoA. These molecules in turn are converted to carnitine conjugates, which can be transported to mitochondria (Wanders and Waterham 2006, Verhoeven et al. 1998, Ferdinandusse et al. 1999).

Literature References
PubMed ID Title Journal Year
9469587 Phytanic acid and pristanic acid are oxidized by sequential peroxisomal and mitochondrial reactions in cultured fibroblasts

Verhoeven, NM, Roe, DS, Kok, RM, Wanders, RJ, Jakobs, C, Roe, CR

J Lipid Res 1998
16756494 Biochemistry of mammalian peroxisomes revisited

Wanders, RJ, Waterham, HR

Annu Rev Biochem 2006
10486279 Molecular cloning and expression of human carnitine octanoyltransferase: evidence for its role in the peroxisomal beta-oxidation of branched-chain fatty acids

Ferdinandusse, S, Mulders, J, IJlst, L, Denis, S, Dacremont, G, Waterham, HR, Wanders, RJ

Biochem Biophys Res Commun 1999
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