D-2-hydroxyglutarate dehydrogenase, mitochondrial (D2HGDH)

Function

• catalyzes the oxidation of D-2-hydroxyglutarate to alpha-ketoglutarate.

(R)-2-hydroxyglutarate + acceptor <-->
             2-oxoglutarate + reduced acceptor

Cofactor:

• FAD
• activated by Zn+2 & Co+

Structure

• belongs to the FAD-binding oxidoreductase/transferase type 4 family

Compartment

mitochondrion

Alternative splicing

named isoforms=2

Pathology

• defects in D2HGDH are the cause of D-2-hydroxyglutaric aciduria

More general terms

• dehydrogenase

Database

• OMIM: https://mirror.omim.org/entry/600721
• UniProt: http://www.uniprot.org/uniprot/Q8N465.html