D-2-hydroxyglutarate dehydrogenase, mitochondrial

Definition:

Catalyzes the oxidation of D-2-hydroxyglutarate (D-2-HG) to alpha-ketoglutarate. Also catalyzes the oxidation of other D-2-hydroxyacids, such as D-malate (D-MAL) and D-lactate (D-LAC). Exhibits high activities towards D-2-HG and D-MAL but a very weak activity towards D-LAC.

References:

[1]. Eduard A Struys, et al. Mutations in phenotypically mild D-2-hydroxyglutaric aciduria. Ann Neurol. 2005 Oct;58(4):626-30. [Content Brief]

[2]. Younes Achouri, et al. Identification of a dehydrogenase acting on D-2-hydroxyglutarate. Biochem J. 2004 Jul 1;381(Pt 1):35-42. [Content Brief]

[3]. Martijn Kranendijk, et al. Evidence for genetic heterogeneity in D-2-hydroxyglutaric aciduria. Hum Mutat. 2010 Mar;31(3):279-83. [Content Brief]

[4]. Eduard A Struys, et al. Mutations in the D-2-hydroxyglutarate dehydrogenase gene cause D-2-hydroxyglutaric aciduria. Am J Hum Genet. 2005 Feb;76(2):358-60. [Content Brief]

[5]. Jun Yang, et al. Structure, substrate specificity, and catalytic mechanism of human D-2-HGDH and insights into pathogenicity of disease-associated mutations. Cell Discov. 2021 Jan 12;7(1):3. [Content Brief]

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