Alpha-oxidation of phytanate

Summary
Organism
Homo sapiens (human)
Reactome
R-HSA-389599
PubChem
R-HSA-389599
Description
  • 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).
Click on a node on the pathway to see its details. Glycoproteins are marked with a glycoprotein icon in their name.
Displaying all 3 entries
UniProt ID Protein Name Gene Symbol Pathway Viewer
O14975 Long-chain fatty acid transport protein 2
  • ACSVL1
  • FACVL1
  • FATP2
  • SLC27A2
  • VLACS
view
Q9BY49 Peroxisomal trans-2-enoyl-CoA reductase
  • PECR
  • PRO1004
  • SDR29C1
view
Q9UJ83 2-hydroxyacyl-CoA lyase 1
  • HACL1
  • HPCL
  • HPCL2
  • HSPC279
  • PHYH2
view
Displaying 1 entry
GlyCosmos Lectin UniProt ID Lectin Name Pathway Viewer
GL_005069 O14832 Phytanoyl-CoA dioxygenase, peroxisomal view

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Acknowledgements

Supported by JST NBDC Grant Number JPMJND2204

Partly supported by NIH Common Fund Grant #1U01GM125267-01


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Last updated: April 6, 2026