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Literature summary for 1.1.5.13 extracted from

  • Rzem, R.; Van Schaftingen, E.; Veiga-da-Cunha, M.
    The gene mutated in L-2-hydroxyglutaric aciduria encodes L-2-hydroxyglutarate dehydrogenase (2006), Biochimie, 88, 113-116.
    View publication on PubMed
Search for more literature for 1.1.5.13

Cloned(Commentary)

Cloned (Comment) Organism
expressed in HEK-293T cells Homo sapiens

Protein Variants

Protein Variants Comment Organism
DELTA-exon9 the mutation leads to severely reduced enzyme activity and is associated with L-2-hydroxyglutaric aciduria Homo sapiens
E176D the mutation leads to severely reduced enzyme activity and is associated with L-2-hydroxyglutaric aciduria Homo sapiens
K81E the mutation leads to severely reduced enzyme activity and is associated with L-2-hydroxyglutaric aciduria Homo sapiens

Inhibitors

Inhibitors Comment Organism Structure
D-2-hydroxyglutarate
-
 Homo sapiens
additional information the activity is not affected by 0.01 mM Co2+, 0.01 mM Zn2+, or 1 mM EDTA Homo sapiens

KM Value [mM]

KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
0.8
-
 L-2-hydroxyglutarate in the absence of FAD, pH and temperature not specified in the publication Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
mitochondrial membrane
-
 Homo sapiens 31966
-

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
L-2-hydroxyglutarate + a quinone Homo sapiens
-
 2-oxoglutarate + a quinol
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
L-2-hydroxyglutarate + a quinone
-
 Homo sapiens 2-oxoglutarate + a quinol
-
 ?

Subunits

Subunits Comment Organism
? x * 45000, SDS-PAGE Homo sapiens

Synonyms

Synonyms Comment Organism
L-2-hydroxyglutarate dehydrogenase
-
 Homo sapiens
lhgO
-
 Homo sapiens
YgaF
-
 Homo sapiens

Cofactor

Cofactor Comment Organism Structure
FAD dependent on Homo sapiens
additional information not stimulated by FMN Homo sapiens

General Information

General Information Comment Organism
malfunction enzyme mutations cause L-2-hydroxyglutaric aciduria Homo sapiens