Gene: [04p153/QDPR] quinoid dihydropteridine reductase; phenylketonuria II, atypical (QDPR/DHPR deficiency);

FUN

Catalyzes reaction: NADPH + 6,7-dihydropteridine = NADP+ + 5,6,7,8-tetrahydropteridine. The substrate is the quinoid form of dihydropteridine which is used as a cofactor in phenylalanine hydroxylation."

FAG

In a functional aspect, quinoid dihydropteridine reductase belongs to the phenylalanine hydroxylase system; on the composition of this system, see GEM:12q241/PAH."

REF

LOC,MOL "Brown RM, Dahl: Genomics, 1, 67-70, 1987
POL,PND,PAT "Dahl HH &: J Med Genet, 25, 25-28, 1988
CLO,SEQ,EXP,POL "Dahl HH &: NAR, 15, 1921-1932, 1987
LOC,MAP "Kuhl P &: Hum Genet, 53, 47-49, 1979
CLO,SEQ,EXP,POL "Lockyer J &: PNAS, 84, N10, 3329-3333, 1987
LOC,MOL "MacDonald ME &: Somat Cell Mol Genet, 13, N5, 569-574, 1987

SWI

SWISSPROT: P09417

KEY

neu, mtbd, aac, vit

CLA

coding, basic

LOC

04 p15.3

MIM

MIM: 261630

EZN

ENZYME: 1.6.99.7

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