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MGP000317

UniProt Annotations

Entry Information
Gene Namebiliverdin reductase A
Protein EntryBIEA_HUMAN
UniProt IDP53004
SpeciesHuman
Comments
Comment typeDescription
Catalytic ActivityBilirubin + NAD(P)(+) = biliverdin + NAD(P)H.
CofactorName=Zn(2+); Xref=ChEBI:CHEBI:29105; Note=Binds 1 zinc ion per subunit.;
DiseaseHyperbiliverdinemia (HBLVD) [MIM:614156]: A condition characterized by a green discoloration of the skin, urine, serum, and other bodily fluids. It is due to increased biliverdin resulting from inefficient conversion to bilirubin. Affected individuals appear to have symptoms only in the context of obstructive cholestasis and/or liver failure. In some cases, green jaundice can resolve after resolution of obstructive cholestasis. {ECO:0000269|PubMed:19580635}. Note=The disease is caused by mutations affecting the gene represented in this entry.
FunctionReduces the gamma-methene bridge of the open tetrapyrrole, biliverdin IX alpha, to bilirubin with the concomitant oxidation of a NADH or NADPH cofactor.
MiscellaneousUses the reactants NADH or NADPH depending on the pH; NADH is used at the acidic pH range (6-6.9) and NADPH at the alkaline range (8.5-8.7). NADPH, however, is the probable reactant in biological systems.
PathwayPorphyrin-containing compound metabolism; protoheme degradation.
SimilarityBelongs to the Gfo/Idh/MocA family. Biliverdin reductase subfamily. {ECO:0000305}.
Subcellular LocationCytoplasm.
SubunitMonomer. {ECO:0000269|Ref.15}.
Tissue SpecificityLiver.
Web ResourceName=NIEHS-SNPs; URL="http://egp.gs.washington.edu/data/blvra/";
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