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Alpha-oxidation of phytanate
Stable Identifier
R-HSA-389599
Type
Pathway
Species
Homo sapiens
Compartment
peroxisomal matrix
ReviewStatus
5/5
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Metabolism (Homo sapiens)
Metabolism of lipids (Homo sapiens)
Fatty acid metabolism (Homo sapiens)
Peroxisomal lipid metabolism (Homo sapiens)
Alpha-oxidation of phytanate (Homo sapiens)
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Phytanic acid arises through ruminant metabolism of chlorophyll and enters the human diet as a constituent of dairy products (Baxter 1968). It can act as an agonist for PPAR and other nuclear hormone receptors, but its normal role in human physiology, if any, is unclear. It is catabolized via a five-step alpha-oxidation reaction sequence that yields pristanoyl-CoA, which is turn is a substrate for beta-oxidation. These reactions take place in the peroxisomal matrix and their failure is associated with Refsum disease (Wanders et al. 2003).
Literature References
PubMed ID
Title
Journal
Year
4177872
Absorption of chlorophyll phytol in normal man and in patients with Refsum's disease
Baxter, JH
J Lipid Res
1968
12633678
Phytanic acid alpha-oxidation, new insights into an old problem: a review
Jansen, GA
,
Wanders, RJA
,
Lloyd, MD
Biochim Biophys Acta
2003
Participants
Events
PMP34-mediated exchange of cytosolic ATP for peroxisomal AMP
(Homo sapiens)
phytanate + CoA-SH + ATP => phytanoyl-CoA + AMP + pyrophosphate
(Homo sapiens)
PECR reduces 2E-phytenoyl-CoA to phytanoyl-CoA
(Homo sapiens)
phytanoyl-CoA + 2-oxoglutarate + O2 => 2-hydroxyphytanoyl-CoA + succinate + CO2
(Homo sapiens)
2-hydroxyphytanoyl-CoA => pristanal + formyl-CoA
(Homo sapiens)
formyl-CoA + H2O => formate + CoASH
(Homo sapiens)
ALDH3A2-2 oxidizes pristanal to pristanate
(Homo sapiens)
pristanate + CoA-SH + ATP => pristanoyl-CoA + AMP + pyrophosphate
(Homo sapiens)
Participates
as an event of
Peroxisomal lipid metabolism (Homo sapiens)
Event Information
Go Biological Process
fatty acid alpha-oxidation (0001561)
Orthologous Events
Alpha-oxidation of phytanate (Bos taurus)
Alpha-oxidation of phytanate (Caenorhabditis elegans)
Alpha-oxidation of phytanate (Canis familiaris)
Alpha-oxidation of phytanate (Danio rerio)
Alpha-oxidation of phytanate (Dictyostelium discoideum)
Alpha-oxidation of phytanate (Drosophila melanogaster)
Alpha-oxidation of phytanate (Gallus gallus)
Alpha-oxidation of phytanate (Mus musculus)
Alpha-oxidation of phytanate (Rattus norvegicus)
Alpha-oxidation of phytanate (Saccharomyces cerevisiae)
Alpha-oxidation of phytanate (Schizosaccharomyces pombe)
Alpha-oxidation of phytanate (Sus scrofa)
Alpha-oxidation of phytanate (Xenopus tropicalis)
Authored
D'Eustachio, P (2009-01-11)
Reviewed
Jassal, B (2009-02-27)
Created
D'Eustachio, P (2009-01-11)
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