CIDeRplusGeneral Information:
| Id: | 7,493 |
| Diseases: |
Metabolic
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| Mammalia | |
| article/cited | |
| Reference: | Milstien S et al.(1996) Purification and cloning of the GTP cyclohydrolase I feedback regulatory protein, GFRP J. Biol. Chem. 271 [PMID: 8702680] |
Interaction Information:
| Comment | GTP cyclohydrolase I is the first and rate-limiting enzyme of the BH4 (6R-L-erythro-5,6,7,8-Tetrahydrobiopterin) biosynthetic pathway. It catalyzes the conversion of GTP into dihydroneopterin triphosphate, the first step of BH4 biosynthesis. Changes in GCH1 activity correlate with changes in BH4 levels. (cited information) |
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Formal Description Interaction-ID: 73935 |
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| Drugbank entries | Show/Hide entries for GCH1 or Tetrahydrobiopterin |
| Comment | A mutation in the GTP cyclohydrolase I gene results in decreased dopamine synthesis and causes a hereditary form of progressive dystonia. (cited information) |
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Formal Description Interaction-ID: 73936 |
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| Drugbank entries | Show/Hide entries for GCH1 or Dopamine |
| Comment | A mutation in the GTP cyclohydrolase I gene results in decreased dopamine synthesis and causes a hereditary form of progressive dystonia. (cited information) |
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Formal Description Interaction-ID: 73937 |
gene/protein affects_activity of |
| Drugbank entries | Show/Hide entries for GCH1 |
| Comment | BH4 level in the plasma of normal children increases after a phenylalanine challenge, but not in children who had a defect in BH4 biosynthesis. This suggests that phenylalanine stimulates BH4 biosynthesis. (cited information) |
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Formal Description Interaction-ID: 73938 |
drug/chemical compound increases_quantity of drug/chemical compound |
| Drugbank entries | Show/Hide entries for |
| Comment | Activity GTP cyclohydrolase I is inhibited by BH4 via end-product feedback regulation. (cited information) |
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Formal Description Interaction-ID: 73939 |
drug/chemical compound decreases_activity of gene/protein |
| Drugbank entries | Show/Hide entries for Tetrahydrobiopterin or GCH1 |
| Comment | Genetic deficiencies of GTP cyclohydrolase I result in hyperphenylalaninemia due to the lack of BH4. (cited information) |
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Formal Description Interaction-ID: 74044 |
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| Drugbank entries | Show/Hide entries for GCH1 |
| Comment | GTP cyclohydrolase I is the first and rate-limiting enzyme of the BH4 (6R-L-erythro-5,6,7,8-Tetrahydrobiopterin) biosynthetic pathway. It catalyzes the conversion of GTP into dihydroneopterin triphosphate, the first step of BH4 biosynthesis. Changes in GCH1 activity correlate with changes in BH4 levels. (cited information) |
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Formal Description Interaction-ID: 74081 |
gene/protein increases_quantity of drug/chemical compound 2-Amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine |
| Drugbank entries | Show/Hide entries for GCH1 |
| Comment | 7,8-Dihydroneopterin 3'-triphosphate (NH2TP) is converted to the unstable intermediate 6-pyruvoyltetrahydropterin (6PPH4). This convertion is catalyzed by catalyzed by 6-pyruvoyltetrahydropterin synthase (6PPH4 synthase). (cited information) |
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Formal Description Interaction-ID: 74094 |
drug/chemical compound 2-Amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine increases_quantity of drug/chemical compound |
| Comment | GTP cyclohydrolase I is the first and rate-limiting enzyme of the BH4 (6R-L-erythro-5,6,7,8-Tetrahydrobiopterin) biosynthetic pathway. It catalyzes the conversion of GTP into dihydroneopterin triphosphate, the first step of BH4 biosynthesis. Changes in GCH1 activity correlate with changes in BH4 levels. (cited information) |
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Formal Description Interaction-ID: 74095 |
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| Drugbank entries | Show/Hide entries for GCH1 |
| Comment | GTP cyclohydrolase I is the first and rate-limiting enzyme of the BH4 (6R-L-erythro-5,6,7,8-Tetrahydrobiopterin) biosynthetic pathway. It catalyzes the conversion of GTP into dihydroneopterin triphosphate, the first step of BH4 biosynthesis. Changes in GCH1 activity correlate with changes in BH4 levels. (cited information) |
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Formal Description Interaction-ID: 74123 |
drug/chemical compound increases_quantity of drug/chemical compound 2-Amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine |
| Comment | 7,8-Dihydroneopterin 3'-triphosphate (NH2TP) is converted to the unstable intermediate 6-pyruvoyltetrahydropterin (6PPH4). This convertion is catalyzed by catalyzed by 6-pyruvoyltetrahydropterin synthase (6PPH4 synthase, PTS). (cited information) |
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Formal Description Interaction-ID: 74127 |
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| Drugbank entries | Show/Hide entries for PTS |
| Comment | 7,8-Dihydroneopterin 3'-triphosphate (NH2TP) is converted to the unstable intermediate 6-pyruvoyltetrahydropterin (6PPH4). This convertion is catalyzed by catalyzed by 6-pyruvoyltetrahydropterin synthase (6PPH4 synthase, PTS). (cited information) |
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Formal Description Interaction-ID: 74128 |
gene/protein decreases_quantity of drug/chemical compound 2-Amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine |
| Drugbank entries | Show/Hide entries for PTS |
| Comment | Sepiapterin reductase catalyzes the convertion of 6-Pyruvoyltetrahydropterin to tetrahydrobiopterin. |
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Formal Description Interaction-ID: 74130 |
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| Drugbank entries | Show/Hide entries for SPR or Tetrahydrobiopterin |
| Comment | Sepiapterin reductase catalyzes the convertion of 6-Pyruvoyltetrahydropterin to tetrahydrobiopterin. |
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Formal Description Interaction-ID: 74132 |
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| Drugbank entries | Show/Hide entries for SPR |
| Comment | Sepiapterin reductase catalyzes the convertion of 6-Pyruvoyltetrahydropterin to tetrahydrobiopterin. |
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Formal Description Interaction-ID: 74133 |
drug/chemical compound increases_quantity of drug/chemical compound |
| Drugbank entries | Show/Hide entries for |