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2-amino-5-formylamino-6-ribofuranosylamino-4(3H)-pyrimidinone triphosphate + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Substrates: substrate is the reaction intermediate of the overall reaction
Products: -
?
beta,gamma-methyleneguanosine 5'-triphosphate + H2O
dihydroneopterin 2',3'-cyclic phosphate + ?
beta-gamma-methyleneguanosine 5'-triphosphate + H2O
beta-gamma-methylene-7,8-dihydroneopterin 3'-triphosphate + formate
-
Substrates: -
Products: -
?
GDP + H2O
dihydroneopterin 2',3'-cyclic phosphate + ?
GDP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine diphosphate
GTP + H2O
2-amino-5-formylamino-6-ribosylamino-4(3H)-pyrimidinone triphosphate + ?
-
Substrates: mutant H179N is not able to perform the whole reaction step
Products: -
r
GTP + H2O
dihydroneopterin triphosphate + formate
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
GTP + H2O
formate + 7,8-dihydro-D-neopterin 2',3'-cyclic phosphate + diphosphate
-
Substrates: -
Products: -
?
GTP + H2O
formate + 7,8-dihydroneopterin 3'-triphosphate
GTP + H2O
formate + D-erythro-dihydroneopterin triphosphate
guanosine 5'-[gamma-thio]triphosphate + H2O
dihydroneopterin 2',3'-cyclic phosphate + ?
-
Substrates: -
Products: -
?
additional information
?
-
beta,gamma-methyleneguanosine 5'-triphosphate + H2O
dihydroneopterin 2',3'-cyclic phosphate + ?
-
Substrates: -
Products: -
?
beta,gamma-methyleneguanosine 5'-triphosphate + H2O
dihydroneopterin 2',3'-cyclic phosphate + ?
-
Substrates: -
Products: -
?
GDP + H2O
dihydroneopterin 2',3'-cyclic phosphate + ?
-
Substrates: -
Products: -
?
GDP + H2O
dihydroneopterin 2',3'-cyclic phosphate + ?
-
Substrates: -
Products: -
?
GDP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine diphosphate
Substrates: -
Products: i.e. dihydroneopterin 3'-triphosphate
?
GDP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine diphosphate
Substrates: -
Products: i.e. dihydroneopterin 3'-triphosphate
?
GTP + H2O
dihydroneopterin triphosphate + formate
-
Substrates: -
Products: -
?
GTP + H2O
dihydroneopterin triphosphate + formate
-
Substrates: first step in the biosynthesis of pteridine coenzymes, such as folic acid and tetrahydrobiopterin
Products: -
?
GTP + H2O
dihydroneopterin triphosphate + formate
-
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Q8S3C2
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Q8S3C2
Substrates: the enzyme mediates the first and committing step pf the pterin branch of the folate-synthesis pathway
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Q8S3C2
Substrates: the enzyme mediates the first and committing step of the pterin branch of the folate-synthesis pathway
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
209541, 209545, 209546, 209547, 209549, 209553, 209563, 209566, 654607, 657176, 670924, 698598 Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Substrates: cyclization to dihydroneopterin triphosphate
Products: i.e. dihydroneopterin triphosphate
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
Substrates: first step in the biosynthesis pathway leading to dihydrofolate and tetrahydrobiopterin
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
Substrates: rate-limiting enzyme in the biosynthesis of tetrahydrobiopterin, important in the regulation of monoamine neurotransmitters such a s dopamine, norepinephrine, and serotonin
Products: -
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GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
Substrates: substrate needs to be Mg2+-free
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Substrates: the enzyme is rate limiting in the tetrahydrobiopterin biosynthesis in adipose tissue, overview
Products: -
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GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
Substrates: the enzyme synthesizes the cofactor for the reaction of the phenylalanine hydroxylase
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Substrates: first committed step in the biosynthesis of tetrahydrofolate and tetrahydrobiopterin
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Substrates: complex series of reaction steps
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Substrates: first committed step in the biosynthesis of tetrahydrofolate and tetrahydrobiopterin
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Substrates: complex series of reaction steps
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
Substrates: the enzyme produces the cofactor tetrahydrobiopterin essential for activities of tyrosine hydroxylase and DOPA decarboxylase
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
209542, 209545, 209546, 209555, 209556, 209557, 209558, 209565, 656261, 667025, 669268, 670416 Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
Substrates: rate-limiting step in the biosynthesis of tetrahydrobiopterin, a key factor necessary for nitric oxide synthase, and for the hydrolxylases that are involved in the production of catecholamines and serotonin
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
Substrates: estrogens play a regulatory role on the enzyme expression, the enzyme is involved in estrogen receptor interaction with cyclic AMP, role of receptor subtypes, overview
Products: -
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GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
Substrates: the enzyme controls the biosynthesis pathway of tetrahydrobiopterin, which is a necessary cofactor for inducible nitric oxide synthase
Products: -
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GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
Substrates: the enzyme is feedback regulated by the GTPCH feedback regulatory protein GFRP, overview
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Serratia indica
-
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Substrates: the enzyme mediates the first and committing step of the pterin branch of the folate-synthesis pathway
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Substrates: -
Products: i.e. dihydroneopterin 3'-triphosphate
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Substrates: -
Products: i.e. dihydroneopterin 3'-triphosphate
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
Substrates: first step in pathway of pterins
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
Substrates: first step in pathway of pterins
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
Substrates: first step in pathway of pterins
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
Substrates: first step in biosynthesis of tetrahydrobiopterin, BH4
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
Substrates: first step in pathway of pterins
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
Substrates: first step in pathway of pterins
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
Substrates: -
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
Substrates: first step in pathway of pterins
Products: -
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
Serratia indica
-
Substrates: first step in pathway of pterins
Products: -
?
GTP + H2O
formate + 7,8-dihydroneopterin 3'-triphosphate
Substrates: -
Products: -
?
GTP + H2O
formate + 7,8-dihydroneopterin 3'-triphosphate
Substrates: -
Products: -
?
GTP + H2O
formate + 7,8-dihydroneopterin 3'-triphosphate
Substrates: -
Products: -
?
GTP + H2O
formate + 7,8-dihydroneopterin 3'-triphosphate
Substrates: -
Products: -
?
GTP + H2O
formate + 7,8-dihydroneopterin 3'-triphosphate
Substrates: -
Products: -
?
GTP + H2O
formate + 7,8-dihydroneopterin 3'-triphosphate
Substrates: -
Products: -
?
GTP + H2O
formate + 7,8-dihydroneopterin 3'-triphosphate
-
Substrates: -
Products: -
?
GTP + H2O
formate + D-erythro-dihydroneopterin triphosphate
-
Substrates: first commited step in the biosynthesis of tetrahydrofolate and tetrahydrobiopterin
Products: -
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GTP + H2O
formate + D-erythro-dihydroneopterin triphosphate
-
Substrates: enzyme catalyzed opening of the imidazole ring of GTP, kinetically competent reaction intermediate is 2-amino-5-formylamino-6-ribosylamino-4(3H)-pyrimidinone
Products: -
?
GTP + H2O
formate + D-erythro-dihydroneopterin triphosphate
-
Substrates: -
Products: -
?
GTP + H2O
formate + D-erythro-dihydroneopterin triphosphate
-
Substrates: first step of the biosynthesis of (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin, i.e. BH4
Products: -
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additional information
?
-
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Substrates: GTPCH1 via tetrahydrobiopterin maintains normal blood pressure and endothelial function in vivo by preserving nitric oxide synthesis by endothelial NO synthase
Products: -
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additional information
?
-
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Substrates: GTP cyclohydrolase I stimulates tyrosine hydroxylase activity by increasing the maximal velocity of the enzyme and fails to block the feedback inhibition of tyrosine hydroxylase by dopamine
Products: -
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additional information
?
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Substrates: GCYH-I is not only the first enzyme of the tetrahydrofolate and tetrahydropterin pathways, but also the first enzyme of queuosine and archaeosine biosynthesis
Products: -
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additional information
?
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Substrates: GCYH-I is not only the first enzyme of the tetrahydrofolate and tetrahydropterin pathways, but also the first enzyme of queuosine and archaeosine biosynthesis
Products: -
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additional information
?
-
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Substrates: GTPCH I is the rate-limiting enzyme for de novo tetrahydrobiopterin synthesis
Products: -
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additional information
?
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Substrates: catalyzes the first step in the biosynthesis of methanopterin
Products: -
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additional information
?
-
-
Substrates: no activity towards dGTP, ATP, GMP, ITP, fapy-GTP, or 7-methyl-GTP
Products: -
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additional information
?
-
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Substrates: catalyzes the first step in the biosynthesis of methanopterin
Products: -
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additional information
?
-
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Substrates: no activity towards dGTP, ATP, GMP, ITP, fapy-GTP, or 7-methyl-GTP
Products: -
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additional information
?
-
-
Substrates: GTPCH1 via tetrahydrobiopterin maintains normal blood pressure and endothelial function in vivo by preserving nitric oxide synthesis by endothelial NO synthase
Products: -
?
additional information
?
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Substrates: tetrahydrobiopterin production by GTPCH I occurs in close proximity to endothelial nitric oxide synthase
Products: -
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additional information
?
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Substrates: isozyme GTPCHIa activity is involved in formation of the black markings on the larva during fourth ecdysis, pigmentation pathway overview
Products: -
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additional information
?
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Substrates: enzyme deficiency causes hyperphenylalaninemia with severe neurological disorders, the 3,4-dihydroxyphenylalanine, i.e. DOPA, responsive form of dystonia, and endothelial dysfunction, all by (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin depletion, the GTP cyclohydrolase I feedback regulatory protein GFRP binds to the enzyme and mediates the regulation of the enzyme by L-phenylalanine and (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin
Products: -
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additional information
?
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Substrates: tetrahydrobiopterin, the cofactor required for hydroxylation of aromatic amino acids, regulates its own synthesis through feedback inhibition of the enzyme mediated by the regulatory subunit called GTP cyclohydrolase I feedback regulatory protein, i.e. GFRP
Products: -
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additional information
?
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Substrates: enzyme is complexed with the GTP cyclohydrolase I feedback regulatory protein, i.e. GFRP, in a ratio of 1:2, complex formation is induced by phenylalanine and (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin
Products: -
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additional information
?
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Substrates: enzyme is complexed with the GTP cyclohydrolase I feedback regulatory protein, i.e. GFRP, in a ratio of 1:2, complex formation is induced by phenylalanine and (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin, i.e. BH4
Products: -
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additional information
?
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Substrates: GCH-1 interacts with many signal transduction proteins such as receptors (NKG2-A/B-actvating NK receptor and latrophilin-2), kinase (Death-associated protein kinase 3), adaptors (Rho-GTPase-activating protein 7 and Rho-guanine nucleotide exchange factor 3) and transcriptional factors (ATF-6 alpha and interferon regulatory factor 1)
Products: -
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additional information
?
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Substrates: GCH1 interacts with GCH1 interacts with GTP cyclohydrolase I feedback regulatory protein, GFRP, and very long-chain specific acyl-CoA dehydrogenase in the liver, tubulin beta-2A chain in the liver and brain, DnaJ homolog subfamily A member 1 and fatty aldehyde dehydrogenase in the liver, heart and kidney and eukaryotic translation initiation factor 3 subunit I in all organs tested, overview. GCH1 associates with mitochondrial proteins and interacts with VLCAD, a mitochondrial protein
Products: -
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additional information
?
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Substrates: GCH1 interacts with GCH1 interacts with GTP cyclohydrolase I feedback regulatory protein, GFRP, and very long-chain specific acyl-CoA dehydrogenase in the liver, tubulin beta-2A chain in the liver and brain, DnaJ homolog subfamily A member 1 and fatty aldehyde dehydrogenase in the liver, heart and kidney and eukaryotic translation initiation factor 3 subunit I in all organs tested, overview. GCH1 associates with mitochondrial proteins and interacts with VLCAD, a mitochondrial protein
Products: -
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