Reactome | (S)-2-hydroxyglutarate + FAD => 2-oxoglutarate + FADH2

(S)-2-hydroxyglutarate + FAD => 2-oxoglutarate + FADH2

Stable Identifier

R-HSA-880050

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial inner membrane, mitochondrial matrix

ReviewStatus

5/5

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(S)-2-hydroxyglutarate + FAD => 2-oxoglutarate + FADH2

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L2HDGH associated with the mitochondrial inner membrane catalyzes the FAD-dependent reaction of (S)-2-hydroxyglutarate to form 2-oxoglutarate (Rzem et al. 2006). L2HDGH mutations are associated with high levels of (S)-2-hydroxyglutarate in vivo and variable neurological symptoms (Rzem et al. 2004; Topcu et al. 2004).

Literature References

PubMed ID	Title	Journal	Year
15548604	A gene encoding a putative FAD-dependent L-2-hydroxyglutarate dehydrogenase is mutated in L-2-hydroxyglutaric aciduria Rzem, R, Veiga-da-Cunha, M, Noel, G, Goffette, S, Nassogne, MC, Tabarki, B, Scholler, C, Marquardt, T, Vikkula, M, Van Schaftingen, E	Proc Natl Acad Sci U S A	2004
15385440	L-2-Hydroxyglutaric aciduria: identification of a mutant gene C14orf160, localized on chromosome 14q22.1 Topçu, M, Jobard, F, Halliez, S, Coskun, T, Yalçinkayal, C, Gerceker, FO, Wanders, RJA, Prud'homme, JF, Lathrop, M, Ozguc, M, Fischer, J	Hum Mol Genet	2004
16005139	The gene mutated in l-2-hydroxyglutaric aciduria encodes l-2-hydroxyglutarate dehydrogenase Rzem, R, Van Schaftingen, E, Veiga-da-Cunha, M	Biochimie	2006