Literature summary for 1.1.99.2 extracted from

Kranendijk, M.; Salomons, G.S.; Gibson, K.M.; Aktuglu-Zeybek, C.; Bekri, S.; Christensen, E.; Clarke, J.; Hahn, A.; Korman, S.H.; Mejaski-Bosnjak, V.; Superti-Furga, A.; Vianey-Saban, C.; van der Knaap, M.S.; Jakobs, C.; Struys, E.A.
Development and implementation of a novel assay for L-2-hydroxyglutarate dehydrogenase (L-2-HGDH) in cell lysates: L-2-HGDH deficiency in 15 patients with L-2-hydroxyglutaric aciduria (2009), J. Inherit. Metab. Dis., 32, 713-719.

Application

Application	Comment	Organism
medicine	an enzyme assay for the determination of L-2-hydroxyglutarate dehydrogenase activity in cells derived from L-2-hydroxyglutaric aciduria patients is developed and implemented	Homo sapiens

KM Value [mM]	KM Value Maximum [mM]	Substrate	Comment	Organism	Structure
additional information	-	L-2-hydroxyglutarate	107 microM/l, lymphoblast	Homo sapiens
additional information	-	L-2-hydroxyglutarate	112 microM/l, fibroblast	Homo sapiens

Localization	Comment	Organism	GeneOntology No.	Textmining
membrane	-	Homo sapiens	16020	-

Organism	UniProt	Comment	Textmining
Homo sapiens	Q9H9P8	-	-

Source Tissue	Comment	Organism	Textmining
fibroblast	-	Homo sapiens	-
lymphoblast	-	Homo sapiens	-
lymphocyte	-	Homo sapiens	-

Substrates	Comment Substrates	Organism	Products	Comment (Products)	Rev.	Reac.
L-2-hydroxyglutarate + iodonitrotetrazolium chloride	activity assay	Homo sapiens	2-oxoglutarate + reduced iodonitrotetrazolium chloride	-	?

Synonyms	Comment	Organism
L-2-HGDH	-	Homo sapiens
L-2-hydroxyglutarate dehydrogenase	-	Homo sapiens

Temperature Optimum [°C]	Temperature Optimum Maximum [°C]	Comment	Organism
37	-	activity assay	Homo sapiens

pH Optimum Minimum	pH Optimum Maximum	Comment	Organism
7	-	activity assay	Homo sapiens

General Information	Comment	Organism
metabolism	L-2-hydroxyglutarate dehydrogenase might be regarded as an enzyme of metabolic repair	Homo sapiens