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MGP004892

UniProt Annotations

Entry Information
Gene Name5-oxoprolinase (ATP-hydrolysing)
Protein EntryOPLA_HUMAN
UniProt IDO14841
SpeciesHuman
Comments
Comment typeDescription
Catalytic ActivityATP + 5-oxo-L-proline + 2 H(2)O = ADP + phosphate + L-glutamate.
Disease5-oxoprolinase deficiency (OPLAHD) [MIM:260005]: A disorder characterized by calcium oxalate/carbonate urolithiasis, and excessive urinary 5-oxo-L-proline. Affected individuals have recurrent episodes of vomiting, diarrhea, and abdominal pain. {ECO:0000269|PubMed:21651516}. Note=The disease is caused by mutations affecting the gene represented in this entry.
FunctionCatalyzes the cleavage of 5-oxo-L-proline to form L- glutamate coupled to the hydrolysis of ATP to ADP and inorganic phosphate.
Sequence CautionSequence=AAB81519.1; Type=Frameshift; Positions=1046, 1068, 1080; Evidence={ECO:0000305}; Sequence=CAB46426.2; Type=Frameshift; Positions=Several; Evidence={ECO:0000305};
SimilarityBelongs to the oxoprolinase family. {ECO:0000305}.
SubunitHomodimer. {ECO:0000250}.
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