Reactome | Beta-oxidation of pristanoyl-CoA

Beta-oxidation of pristanoyl-CoA

Stable Identifier

R-HSA-389887

Type

Pathway

Species

Homo sapiens

Compartment

peroxisomal matrix

ReviewStatus

5/5

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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
16756494	Biochemistry of mammalian peroxisomes revisited Wanders, RJA, Waterham, HR	Annu Rev Biochem	2006
9469587	Phytanic acid and pristanic acid are oxidized by sequential peroxisomal and mitochondrial reactions in cultured fibroblasts Verhoeven, NM, Roe, DS, Kok, RM, Wanders, RJA, Jakobs, C, Roe, CR	J Lipid Res	1998
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, RJA	Biochem Biophys Res Commun	1999