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Literature summary for 1.14.16.1 extracted from

  • Pey, A.L.; Martinez, A.; Charubala, R.; Maitland, D.J.; Teigen, K.; Calvo, A.; Pfleiderer, W.; Wood, J.M.; Schallreuter, K.U.
    Specific interaction of the diastereomers 7(R)- and 7(S)-tetrahydrobiopterin with phenylalanine hydroxylase: implications for understanding primapterinuria and vitiligo (2006), FASEB J., 20, 2130-2132.
    View publication on PubMed
Search for more literature for 1.14.16.1

Inhibitors

Inhibitors Comment Organism Structure
(7R)-tetrahydrobiopterin slight inhibition, synthetic pathway, overview, conformational structure by NMR Homo sapiens
(7S)-tetrahydrobiopterin strong, competitive inhibition, synthetic pathway, overview, conformational structure by NMR Homo sapiens
7(S)-tetrahydrobiopterin
-
 Homo sapiens

KM Value [mM]

KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
additional information
-
 additional information kinetics, molecular dynamics simulations, structure-energetics calculations, and molecular interaction fields, overview Homo sapiens
0.22
-
 7(R,S)-tetrahydrobiopterin pH 7.0, 25°C, recombinant enzyme Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
L-phenylalanine + (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + O2 Homo sapiens
-
 L-tyrosine + 4a-hydroxy-(6R)-L-erythro-5,6,7,8-tetrahydrobiopterin
-
 ?
additional information Homo sapiens pterin-4a-carbinolamine dehydratase, PCD, is an essential component of the phenylalanine hydroxylase system, catalyzing the regeneration of the essential cofactor (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin, i.e. (6R)BH4, PHA is implicated in primapterinuria, a variant form of phenylketonuria, phenylalanine hydroxylase system overview ?
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens P00439
-
-

Reaction

Reaction Comment Organism Reaction ID
L-phenylalanine + a 5,6,7,8-tetrahydropteridine + O2 = L-tyrosine + a 4a-hydroxy-5,6,7,8-tetrahydropteridine kinetics, molecular dynamics simulations, structure-energetics calculations, and molecular interaction fields, structure-function analysis Homo sapiens
a

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
L-phenylalanine + (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + O2
-
 Homo sapiens L-tyrosine + 4a-hydroxy-(6R)-L-erythro-5,6,7,8-tetrahydrobiopterin
-
 ?
L-phenylalanine + (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + O2 structure-function relationships Homo sapiens L-tyrosine + 4a-hydroxy-(6R)-L-erythro-5,6,7,8-tetrahydrobiopterin
-
 ?
L-phenylalanine + (7R,S)-tetrahydrobiopterin + O2
-
 Homo sapiens L-tyrosine + 4a-hydroxytetrahydrobiopterin
-
 r
additional information pterin-4a-carbinolamine dehydratase, PCD, is an essential component of the phenylalanine hydroxylase system, catalyzing the regeneration of the essential cofactor (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin, i.e. (6R)BH4, PHA is implicated in primapterinuria, a variant form of phenylketonuria, phenylalanine hydroxylase system overview Homo sapiens ?
-
 ?

Subunits

Subunits Comment Organism
tetramer
-
 Homo sapiens

Synonyms

Synonyms Comment Organism
PAH
-
 Homo sapiens
phenylalanine hydroxylase
-
 Homo sapiens

Temperature Optimum [°C]

Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
25
-
 assay at Homo sapiens

pH Optimum

pH Optimum Minimum pH Optimum Maximum Comment Organism
7
-
 assay at Homo sapiens

Cofactor

Cofactor Comment Organism Structure
(6R)-L-erythro-5,6,7,8-tetrahydrobiopterin i.e. 6(R)BH4, essential cofactor Homo sapiens
(7R)-tetrahydrobiopterin 8fold lower affinity and activity compared with 6(R)BH4 Homo sapiens
(7S)-tetrahydrobiopterin 8fold lower affinity and activity compared with 6(R)BH4 Homo sapiens
additional information cofactor binding analysis, overview Homo sapiens

Ki Value [mM]

Ki Value [mM] Ki Value maximum [mM] Inhibitor Comment Organism Structure
additional information
-
 additional information inhibition kinetics Homo sapiens
0.0023 0.0049 7(S)-tetrahydrobiopterin pH 7.0, 25°C, recombinant enzyme, versus (6R)-tetrahydrobiopterin Homo sapiens