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1.1.99.39: D-2-hydroxyglutarate dehydrogenase

This is an abbreviated version!
For detailed information about D-2-hydroxyglutarate dehydrogenase, go to the full flat file.

Word Map on EC 1.1.99.39

Reaction

(R)-2-hydroxyglutarate
+
acceptor
=
2-oxoglutarate
 +
reduced acceptor

Synonyms

2-hydroxyglutarate dehydrogenase, AtD-2HGDH, D-2-HG dehydrogenase, D-2-HGA, D-2-hydroxyglutarate dehydrogenase, D2-hydroxyglutarate dehydrogenase, D2HDH, D2HGDH

ECTree

     1 Oxidoreductases
         1.1 Acting on the CH-OH group of donors
             1.1.99 With unknown physiological acceptors
                1.1.99.39 D-2-hydroxyglutarate dehydrogenase

Natural Substrates Products

Natural Substrates Products on EC 1.1.99.39 - D-2-hydroxyglutarate dehydrogenase

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NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
(R)-2-hydroxyglutarate + acceptor
2-oxoglutarate + reduced acceptor
show the reaction diagram
(R)-2-hydroxyglutarate + electron transfer flavoprotein
2-oxoglutarate + reduced electron transfer flavoprotein
show the reaction diagram
(S)-2-hydroxyglutarate + acceptor
2-oxoglutarate + reduced acceptor
show the reaction diagram
necessary step in the serine biosynthetic pathway
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-
?
additional information
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description of two pathogenic mutations in the D-2-hydroxyglutarate dehydrogenase gene causing D-2-HGA with a very mild clinical presentation: a splice error (IVS4-2A->G) and a missense mutation (c.1315A->G, p.Asn439Asp). Mutations in the D-2-hydroxyglutarate dehydrogenase gene cause bith the severe and mild phenotypes of D-2-HGA
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?