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Literature summary 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.
    View publication on PubMed

Application

EC Number Application Comment Organism
1.1.99.2 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]

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

Localization

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

Organism

EC Number Organism UniProt Comment Textmining
1.1.99.2 Homo sapiens Q9H9P8
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
1.1.99.2 fibroblast
-
 Homo sapiens
-
1.1.99.2 lymphoblast
-
 Homo sapiens
-
1.1.99.2 lymphocyte
-
 Homo sapiens
-

Substrates and Products (Substrate)

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

Synonyms

EC Number Synonyms Comment Organism
1.1.99.2 L-2-HGDH
-
 Homo sapiens
1.1.99.2 L-2-hydroxyglutarate dehydrogenase
-
 Homo sapiens

Temperature Optimum [°C]

EC Number Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
1.1.99.2 37
-
 activity assay Homo sapiens

pH Optimum

EC Number pH Optimum Minimum pH Optimum Maximum Comment Organism
1.1.99.2 7
-
 activity assay Homo sapiens

General Information

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