dihydropteridine reductase (HDHPR, quinoid dihydropteridine reductase, QDPR, DHPR)
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Function
- product of this enzyme, tetrahydrobiopterin (BH-4), is an essential cofactor for
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
More general terms
References
- ↑ OMIM https://mirror.omim.org/entry/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 http://www.uniprot.org/uniprot/P09417.html