Glyoxylate metabolism and glycine degradation

Summary
Organism
Homo sapiens (human)
Reactome
R-HSA-389661
PubChem
R-HSA-389661
Description
  • Glyoxylate is generated in the course of glycine and hydroxyproline catabolism and can be converted to oxalate. In humans, this process takes place in the liver. Defects in two enzymes of glyoxylate metabolism, alanine:glyoxylate aminotransferase (AGXT) and glycerate dehydrogenase/glyoxylate reductase (GRHPR), are associated with pathogenic overproduction of oxalate (Danpure 2005). The reactions that interconvert glycine, glycolate, and glyoxylate and convert glyoxylate to oxalate have been characterized in molecular detail in humans. A reaction sequence for the conversion of hydroxyproline to glyoxylate has been inferred from studies of partially purified extracts of rat and bovine liver but the enzymes involved in the corresponding human reactions have not been identified.
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
Q86XE5 4-hydroxy-2-oxoglutarate aldolase, mitochondrial
  • C10orf65
  • DHDPSL
  • HOGA1
view
Q9UBQ7 Glyoxylate reductase/hydroxypyruvate reductase
  • GLXR
  • GRHPR
  • MSTP035
view
Q9UF12 Hydroxyproline dehydrogenase
  • HSPOX1
  • HYPDH
  • PRODH2
view

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International Collaboration

GlyCosmos is a member of the GlySpace Alliance together with GlyGen and Glycomics@ExPASy.

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