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Literature summary for 3.5.4.16 extracted from

  • Christensen, R.; Alhonen, L.; Wahlfors, J.; Jakobsen, M.; Jensen, T.G.
    Characterization of transgenic mice with the expression of phenylalanine hydroxylase and GTP cyclohydrolase I in the skin (2005), Exp. Dermatol., 14, 535-542.
    View publication on PubMed

Application

Application Comment Organism
biotechnology creation of a phenylalanine sink by transgenic overexpression of phenylalnine hydroxylase and GTP cyclohydrolase I in the skin to reduce high phenylalanine concentrations in the blood in phenylketonuria metabolic disease Mus musculus

Protein Variants

Protein Variants Comment Organism
additional information construction of transgenic mice overexpressing the enzyme specifically in the skin together with phenylalanine hydroxylase, the clearance of phenylalanine from blood is increased in transgenic mice, measured in those with phenylketonuria, mice genotyping, overview Mus musculus

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
GTP + H2O Mus musculus the enzyme synthesizes the cofactor for the reaction of the phenylalanine hydroxylase formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
?

Organism

Organism UniProt Comment Textmining
Mus musculus
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
GTP + H2O
-
Mus musculus formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
?
GTP + H2O the enzyme synthesizes the cofactor for the reaction of the phenylalanine hydroxylase Mus musculus formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
?

Synonyms

Synonyms Comment Organism
GTP-CH
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Mus musculus