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dihydropteridine reductase (HDHPR, quinoid dihydropteridine reductase, QDPR, DHPR)
Function:
- product of this enzyme, tetrahydrobiopterin (BH-4), is an essential cofactor for
a) phenylalanine hydroxylase
b) tyrosine hydroxylase
c) tryptophan hydroxylase
5,6,7,8-tetrahydropteridine + NAD+/NADP+
6,7-dihydropteridine + NADH/NADPH
Inhibited by trimethoprim
Structure:
- homodimer
- belongs to the short-chain dehydrogenases/reductases (SDR) family
Pathology:
- defects in QDPR are the cause of phenylketonuria 2
Laboratory:
- dihydropteridine reductase in erythrocytes
General
oxidoreductase
Properties
MOTIF: active site
MISC-INFO: INHIBITOR = TRIMETHOPRIM
Database Correlations
OMIM 261630
UniProt P09417
Pfam PF00106
Kegg hsa:5860
ENZYME 1.5.1.34
References
- OMIM :accession 261630
- BIOMDB: Db of mutations causing tetrahydrobiopterin
deficiencies
http://www.bh4.org/biodef1.html
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=QDPR
- UniProt :accession P09417