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Literature summary extracted from

  • Hariharan, V.A.; Denton, T.T.; Paraszcszak, S.; McEvoy, K.; Jeitner, T.M.; Krasnikov, B.F.; Cooper, A.J.
    The enzymology of 2-hydroxyglutarate, 2-hydroxyglutaramate and 2-hydroxysuccinamate and their relationship to oncometabolites (2017), Biology, 6, E24 .
    View publication on PubMedView publication on EuropePMC

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
1.1.99.2 L-2-hydroxyglutarate + FAD Homo sapiens
-
 2-oxoglutarate + FADH2
-
 ir
1.1.99.2 L-2-hydroxyglutarate + NAD+ Homo sapiens
-
 2-oxoglutarate + NADH + H+
-
 r

Organism

EC Number Organism UniProt Comment Textmining
1.1.99.2 Homo sapiens
-
-
-
3.5.1.3 Homo sapiens Q9NQR4
-
-

Specific Activity [micromol/min/mg]

EC Number Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
3.5.1.3 0.51
-
 substrate: L-2-hydroxysuccinamate, pH 7.4, 37°C Homo sapiens
3.5.1.3 1.4
-
 substrate: L-2-hydroxyglutaramate, pH 7.4, 37°C Homo sapiens
3.5.1.3 3
-
 substrate: 2-oxoglutaramate, pH 7.4, 37°C Homo sapiens
3.5.1.3 3.4
-
 substrate: succinamate, pH 7.4, 37°C Homo sapiens

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.1.99.2 L-2-hydroxyglutarate + FAD
-
 Homo sapiens 2-oxoglutarate + FADH2
-
 ir
1.1.99.2 L-2-hydroxyglutarate + NAD+
-
 Homo sapiens 2-oxoglutarate + NADH + H+
-
 r
3.5.1.3 2-oxoglutaramate + H2O
-
 Homo sapiens 2-oxoglutarate + NH3
-
 ?
3.5.1.3 2-oxosuccinamate + H2O
-
 Homo sapiens oxaloacetate + NH3
-
 ?
3.5.1.3 L-2-hydroxyglutaramate + H2O
-
 Homo sapiens L-2-hydroxyglutarate + NH3
-
 ?
3.5.1.3 L-2-hydroxysuccinamate + H2O
-
 Homo sapiens L-malate + NH3
-
 ?

Synonyms

EC Number Synonyms Comment Organism
1.1.99.2 L-2-HG dehydrogenase
-
 Homo sapiens
3.5.1.3 NIT2
-
 Homo sapiens
3.5.1.3 nitrilase-like protein 2
-
 Homo sapiens
3.5.1.3 omega-amidase
-
 Homo sapiens
3.5.1.3 omega-amidodicarboxylate amidohydrolase
-
 Homo sapiens

Temperature Optimum [°C]

EC Number Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
3.5.1.3 37
-
 assay at Homo sapiens

pH Optimum

EC Number pH Optimum Minimum pH Optimum Maximum Comment Organism
3.5.1.3 8
-
 assay at Homo sapiens

General Information

EC Number General Information Comment Organism
1.1.99.2 malfunction L-2-hydroxyglutaric aciduria is a rare inborn error of metabolism in the enzyme is defective Homo sapiens
3.5.1.3 metabolism the enzyme may be regarded as a repair enzyme for salvaging L-2-hydroxysuccinamate (as L-malate), and, working in conjunction with L-2-hydroxyglutarate dehydrogenase, for salvaging L-2-hydroxyglutaramate (as 2-oxoglutarate) Homo sapiens