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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General Information
General Information on EC 1.14.16.1 - phenylalanine 4-monooxygenase
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malfunction
physiological function
phenylketonuria is an autosomal recessive metabolic disorder caused by a deficiency of phenylalanine hydroxylase
malfunction
phenylketonuria results from defects in phenylalanine hydroxylase
malfunction
a pah null mutant and an episomal complemented overexpressing derivative (pah-/+PAH) are generated. Wild-type but not null mutants parasites show reactivity with an antibody to melanin when grown with l-3,4-dihydroxyphenylalanine. Wild-type is auxotrophic for Phe, Trp and Tyr, suggesting that PAH activity is insufficient to meet normal Tyr requirements. PAH null mutants show an increased sensitivity to Tyr deprivation, while the pah-/+PAH overexpressor shows increased survival and can be adapted to grow well without added Tyr. PAH null mutant shows no alterations in tetrahydrobiopterin-dependent differentiation
malfunction
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dysfunctional phenylalanine hydroxylase leads to phenylketonuria
malfunction
dysfunctional phenylalanine hydroxylase leads to phenylketonuria
malfunction
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missense mutations of the enzyme are associated with the monogenic disease phenylketonuria
malfunction
phenylalanine hydroxylase dysfunction causes phenylketonuria
malfunction
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dysfunctional phenylalanine hydroxylase leads to phenylketonuria
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key enzyme in the sulfoxidation of S-carboxymethyl-L-cysteine S-oxide and its thioester metabolites S-methyl-L-cysteine, N-acetyl-S-carboxymethyl-L-cysteine, and N-acetyl-S-methyl-L-cysteine
physiological function
key enzyme in the sulfoxidation of S-carboxymethyl-L-cysteine S-oxide and its thioester metabolites S-methyl-L-cysteine, N-acetyl-S-carboxymethyl-L-cysteine, and N-acetyl-S-methyl-L-cysteine
physiological function
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the enzyme has a major functional role in the synthesis of pyomelanin and promotes growth in media lacking tyrosine
physiological function
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the enzyme has a major functional role in the synthesis of pyomelanin and promotes growth in media lacking tyrosine
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