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

Stable Identifier
R-HSA-880002
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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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
3182820 Isolation and characterization of a hydroxyacid-oxoacid transhydrogenase from rat kidney mitochondria

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

J Biol Chem 1988
16435184 Kinetic characterization of human hydroxyacid-oxoacid transhydrogenase: relevance to D-2-hydroxyglutaric and gamma-hydroxybutyric acidurias

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

J Inherit Metab Dis 2005
Participants
Participant Of
Event Information
Catalyst Activity
Catalyst Activity
Title
hydroxyacid-oxoacid transhydrogenase activity of ADHFE1(1-?) [mitochondrial matrix]
Physical Entity
Activity
Orthologous Events
Authored
Reviewed
Created