QDPR Chromosome 4

Quinoid dihydropteridine reductase
54 variants 54 Health Risk

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What This Gene Does
This gene encodes the enzyme dihydropteridine reductase, which catalyzes the NADH-mediated reduction of quinonoid dihydrobiopterin. This enzyme is an essential component of the pterin-dependent aromatic amino acid hydroxylating systems. Mutations in this gene resulting in QDPR deficiency include aberrant splicing, amino acid substitutions, insertions, or premature terminations. Dihydropteridine reductase deficiency presents as atypical phenylketonuria due to insufficient production of biopterin, a cofactor for phenylalanine hydroxylase. [provided by RefSeq, Jul 2008]
Gene Info
Gene Group
Short chain dehydrogenase/reductase superfamily
Locus Type
gene with protein product
Location
4p15.32
Ensembl
ENSG00000151552
Associated Conditions (6)
Dihydropteridine reductase deficiency
Inborn genetic diseases
QDPR-related disorder
6-Pyruvoyl-tetrahydrobiopterin synthase deficiency
Colorectal cancer
Hyperphenylalaninemia due to tetrahydrobiopterin deficiency
Key Variants
RS112209600
Conflicting classifications of pathogenicity
Dihydropteridine reductase deficiency, Inborn genetic diseases, Dihydropteridine reductase deficiency
Health Risk
RS1309099253
Conflicting classifications of pathogenicity
Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
Health Risk
RS140949360
Conflicting classifications of pathogenicity
Dihydropteridine reductase deficiency, QDPR-related disorder, Dihydropteridine reductase deficiency
Health Risk
RS754982401
Conflicting classifications of pathogenicity
Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
Health Risk
RS758659025
Conflicting classifications of pathogenicity
Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
Health Risk
RS762480653
Conflicting classifications of pathogenicity
Dihydropteridine reductase deficiency, Inborn genetic diseases, Dihydropteridine reductase deficiency
Health Risk
RS776479029
Conflicting classifications of pathogenicity
Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
Health Risk
RS1028029163
Likely pathogenic
Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
Health Risk
RS104893865
Likely pathogenic
Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
Health Risk
RS1184906056
Likely pathogenic
Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
Health Risk
RS1364780551
Likely pathogenic
Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
Health Risk
RS1428018512
Likely pathogenic
Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
Health Risk
All Variants (54)
RSID Category Clinical Significance Conditions
RS112209600 Health Risk Conflicting classifications of pathogenicity Dihydropteridine reductase deficiency, Inborn genetic diseases, Dihydropteridine reductase deficiency
RS1309099253 Health Risk Conflicting classifications of pathogenicity Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS140949360 Health Risk Conflicting classifications of pathogenicity Dihydropteridine reductase deficiency, QDPR-related disorder, Dihydropteridine reductase deficiency
RS754982401 Health Risk Conflicting classifications of pathogenicity Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS758659025 Health Risk Conflicting classifications of pathogenicity Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS762480653 Health Risk Conflicting classifications of pathogenicity Dihydropteridine reductase deficiency, Inborn genetic diseases, Dihydropteridine reductase deficiency
RS776479029 Health Risk Conflicting classifications of pathogenicity Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS1028029163 Health Risk Likely pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS104893865 Health Risk Likely pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS1184906056 Health Risk Likely pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS1364780551 Health Risk Likely pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS1428018512 Health Risk Likely pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS1444280419 Health Risk Likely pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS1577184852 Health Risk Likely pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS2474671527 Health Risk Likely pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS2474684351 Health Risk Likely pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS569310979 Health Risk Likely pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS750513275 Health Risk Likely pathogenic
RS762772317 Health Risk Likely pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS776171302 Health Risk Likely pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS777797545 Health Risk Likely pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS995264302 Health Risk Likely pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS104893864 Health Risk Pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS104893866 Health Risk Pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS104893867 Health Risk Pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS1171544975 Health Risk Pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS1262627752 Health Risk Pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS1362475419 Health Risk Pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS1407920390 Health Risk Pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS1577191558 Health Risk Pathogenic Dihydropteridine reductase deficiency, 6-Pyruvoyl-tetrahydrobiopterin synthase deficiency, Dihydropteridine reductase deficiency
RS1577195951 Health Risk Pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS2108987681 Health Risk Pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS2108987721 Health Risk Pathogenic
RS2108987974 Health Risk Pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS2108992958 Health Risk Pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS2108993008 Health Risk Pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS2108997263 Health Risk Pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS2108999089 Health Risk Pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS2474656264 Health Risk Pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS2474656670 Health Risk Pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS2474658353 Health Risk Pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS2474680455 Health Risk Pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS761619802 Health Risk Pathogenic Dihydropteridine reductase deficiency, Colorectal cancer, Dihydropteridine reductase deficiency
RS769460415 Health Risk Pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS778806991 Health Risk Pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS779997983 Health Risk Pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS1034528249 Health Risk Pathogenic/Likely pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
RS104893863 Health Risk Pathogenic/Likely pathogenic Dihydropteridine reductase deficiency, Hyperphenylalaninemia due to tetrahydrobiopterin deficiency, Dihydropteridine reductase deficiency
RS1254995369 Health Risk Pathogenic/Likely pathogenic QDPR-related disorder, Dihydropteridine reductase deficiency, QDPR-related disorder
RS1718196012 Health Risk Pathogenic/Likely pathogenic Dihydropteridine reductase deficiency, Dihydropteridine reductase deficiency
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