D2HGDH : D-2-hydroxyglutarate dehydrogenase
Description
The D2HGDH (D-2-hydroxyglutarate dehydrogenase) is a protein-coding gene located on chromosome 2.
The D2HGDH gene provides instructions for making an enzyme called D-2-hydroxyglutarate dehydrogenase. This enzyme is found in mitochondria, which are the energy-producing centers within cells. Within mitochondria, the enzyme participates in reactions that produce energy for cell activities. Specifically, D-2-hydroxyglutarate dehydrogenase converts a compound called D-2-hydroxyglutarate to another compound called 2-ketoglutarate. A series of additional enzymes further process 2-ketoglutarate to produce energy.
The D2HGDH gene encodes for an enzyme, D-2-hydroxyglutarate dehydrogenase, which catalyzes the oxidation of D-2-hydroxyglutarate (D-2-HG) to alpha-ketoglutarate. This enzyme also exhibits activity towards other D-2-hydroxyacids such as D-malate (D-MAL) and D-lactate (D-LAC), but with a much weaker activity towards D-LAC compared to D-2-HG and D-MAL.
D2HGDH is also known as D2HGD.
