1.14.16.1: phenylalanine 4-monooxygenase
This is an abbreviated version!
For detailed information about phenylalanine 4-monooxygenase, go to the full flat file.
Word Map on EC 1.14.16.1
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1.14.16.1
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phenylketonuria
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hyperphenylalaninemia
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bh4
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error
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pterins
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inborn
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children
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hydroxylases
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neurotransmitter
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province
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tetrahydropterins
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counsel
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intellectual
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dopamine
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l-tyrosine
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genotype-phenotype
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prenatal
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serotonin
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dihydropteridine
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caucasian
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catecholamine
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hepatocytes
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sepiapterin
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genotype-based
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quinonoid
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non-heme
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chromobacterium
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neopterin
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ligation-dependent
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dihydrochloride
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neuropsychological
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lysolecithin
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lifelong
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phenylpyruvate
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dopa
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cyclohydrolase
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molecular biology
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rflps
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p-chlorophenylalanine
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dihydrobiopterin
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hypopigmentation
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s-oxidation
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pteridine
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violaceum
- 1.14.16.1
- phenylketonuria
- hyperphenylalaninemia
- bh4
- error
- pterins
-
inborn
- children
- hydroxylases
-
neurotransmitter
-
province
- tetrahydropterins
-
counsel
-
intellectual
- dopamine
- l-tyrosine
-
genotype-phenotype
-
prenatal
- serotonin
- dihydropteridine
-
caucasian
- catecholamine
- hepatocytes
- sepiapterin
-
genotype-based
-
quinonoid
-
non-heme
-
chromobacterium
- neopterin
-
ligation-dependent
- dihydrochloride
-
neuropsychological
- lysolecithin
-
lifelong
- phenylpyruvate
- dopa
-
cyclohydrolase
- molecular biology
-
rflps
- p-chlorophenylalanine
- dihydrobiopterin
-
hypopigmentation
-
s-oxidation
- pteridine
- violaceum
Reaction
Synonyms
cePAH, DicPAH, EC 1.14.3.1, EC 1.99.1.2, HPAH, L-phenylalanine 4-hydroxylase, oxygenase, phenylalanine 4-mono-, P4H, PAH, PheH, phenylalaninase, phenylalanine 4-hydroxylase, phenylalanine hydroxylase, phenylalanine monooxygenase, PheOH, phhA
ECTree
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Cofactor
Cofactor on EC 1.14.16.1 - phenylalanine 4-monooxygenase
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(6R)-5,6,7,8-tetrahydro-L-monapterin
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7% of the activity with tetrahydrobiopterin
(7R)-tetrahydrobiopterin
8fold lower affinity and activity compared with 6(R)BH4
(7S)-tetrahydrobiopterin
8fold lower affinity and activity compared with 6(R)BH4
6,7-dimethyltetrahydropterin
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17% of the activity with tetrahydrobiopterin
(6R)-L-erythro-5,6,7,8-tetrahydrobiopterin
i.e. 6(R)BH4, essential cofactor
5,6,7,8-tetrahydro-L-biopterin
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PAH can be activated up to 20times in the presence of its natural cofactor 5,6,7,8-tetrahydro-L-biopterin but cannot be activated in the presence of synthetic cofactors such as 6,7-dimethyltetrahydrobiopterin or 6-methyltetrahydropterin
tetrahydrobiopterin
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tetrahydrobiopterin
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tetrahydrobiopterin
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required for activity, binding involves Ph254 and Tyr325, structure overview
tetrahydrofolate
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10% of activity with 6,7-dimethyltetrahydropterin
tetrahydrofolate
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20% of the activity with tetrahydrobiopterin
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artificial cofactors: 6-methyltetrahydropterin, 6,7-dimethyltetrahydropterin
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additional information
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artificial cofactors: 6-methyltetrahydropterin, 6,7-dimethyltetrahydropterin
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additional information
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artificial cofactors: 6-methyltetrahydropterin, 6,7-dimethyltetrahydropterin
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additional information
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artificial cofactors: 6-methyltetrahydropterin, 6,7-dimethyltetrahydropterin
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additional information
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artificial cofactors: 6-methyltetrahydropterin, 6,7-dimethyltetrahydropterin
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additional information
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artificial cofactor: 2,4,5-triamino-6-hydroxypyrimidine
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additional information
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artificial cofactors: 6-methyltetrahydropterin, 7-methylpterin
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additional information
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artificial cofactors: 6-methyltetrahydropterin, 7-methylpterin
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additional information
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artificial cofactors: 2-amino-4-hydroxy-6,7-dimethyltetrahydropteridine, 6-methyl-5-deazatetrahydropterin and 2-amino-4-hydroxy-6-methyltetrahydropteridine can replace tetrahydrobiopterin
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additional information
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artificial cofactors: 2-amino-4-hydroxy-6,7-dimethyltetrahydropteridine, 6-methyl-5-deazatetrahydropterin and 2-amino-4-hydroxy-6-methyltetrahydropteridine can replace tetrahydrobiopterin
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