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