(R)-2-hydroxyglutarate + succinate semialdehyde <=> 2-oxoglutarate + 4-hydroxybutyrate

Stable Identifier
R-HSA-880002
Type
Reaction [transition]
Species
Homo sapiens
Compartment
ReviewStatus
5/5
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ADHFE1 catalyzes the reversible reaction of (R)-2-hydroxyglutarate and succinate semialdehyde to form 2-oxoglutarate and 4-hydroxybutyrate (Struys et al. 2005). The localization of human ADHFE1 to the mitochondrial matrix is inferred from the location determined experimentally for its rat homolog (Kaufman et al. 1988).
Literature References
PubMed ID Title Journal Year
16435184 Kinetic characterization of human hydroxyacid-oxoacid transhydrogenase: relevance to D-2-hydroxyglutaric and gamma-hydroxybutyric acidurias

Verhoeven, NM, Wickenhagen, WV, Ten Brink, HJ, Gibson, KM, Struys, EA, Jakobs, C

J Inherit Metab Dis 2005
3182820 Isolation and characterization of a hydroxyacid-oxoacid transhydrogenase from rat kidney mitochondria

Levin, DM, Fales, HM, Nelson, T, Kaufman, EE

J Biol Chem 1988
Participants
Participates
Event Information
Catalyst Activity

hydroxyacid-oxoacid transhydrogenase activity of ADHFE1(1-?) [mitochondrial matrix]

Orthologous Events
Cross References
Rhea
Authored
Reviewed
Created
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