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Literature summary for 3.5.2.9 extracted from

  • Almaghlouth, I.; Mohamed, J.; Al-Amoudi, M.; Al-Ahaidib, L.; Al-Odaib, A.; Alkuraya, F.
    5-Oxoprolinase deficiency: report of the first human OPLAH mutation (2012), Clin. Genet., 82, 193-196.
    View publication on PubMed

Metals/Ions

Metals/Ions Comment Organism Structure
Mg2+ required Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
ATP + 5-oxo-L-proline + 2 H2O Homo sapiens
-
ADP + phosphate + L-glutamate
-
?

Organism

Organism UniProt Comment Textmining
Homo sapiens O14841
-
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
ATP + 5-oxo-L-proline + 2 H2O
-
Homo sapiens ADP + phosphate + L-glutamate
-
?

Synonyms

Synonyms Comment Organism
5-oxoprolinase
-
Homo sapiens
OPLAH
-
Homo sapiens

Cofactor

Cofactor Comment Organism Structure
ATP
-
Homo sapiens

General Information

General Information Comment Organism
malfunction inherited 5-oxoprolinase deficiency is a rare 5-oxoprolinase deficiency is an extremely rare disorder of the gamma-glutamyl cycle characterised by 5-oxoprolinuria, heterogeneity of the clinical presentation which ranges from normal to significant neurological involvement, genotype-phenotype correlation, phenotypes, overview Homo sapiens
metabolism the enzyme is involved in the gamma-glutamyl cycle, a six-enzyme cycle that represents the primary pathway for glutathione synthesis and degradation Homo sapiens