1.14.16.1: phenylalanine 4-monooxygenase

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For detailed information about phenylalanine 4-monooxygenase, go to the full flat file.

Word Map on EC 1.14.16.1

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

a 4a-hydroxy-5,6,7,8-tetrahydropteridine

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

1 Oxidoreductases

1.14 Acting on paired donors, with incorporation or reduction of molecular oxygen

1.14.16 With reduced pteridine as one donor, and incorporation of one atom of oxygen into the other donor

1.14.16.1 phenylalanine 4-monooxygenase

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Results	in table
71	Activating Compound
7761	AA Sequence
2	Application
1	CAS Registry Number
66	Cloned(Commentary)
95	Cofactor
12	Crystallization (Commentary)
2337	Disease/ Diagnostics
1	Expression
13	General Information
5	General Stability
1	IC50 Value
98	Inhibitors
14	kcat/KM [mM/s]
24	Ki Value [mM]
309	KM Value [mM]
10	Localization
52	Metals/Ions
55	Molecular Weight [Da]
81	Natural Substrates/ Products (Substrates)
349	Organism
9	Pathway
73	PDB ID
14	pH Optimum
1	pH Range
13	Posttranslational Modification
452	Protein Variants
50	Purification (Commentary)
17	Reaction
6	Reaction Type
175	Reference
44	Source Tissue
127	Specific Activity [micromol/min/mg]
5	Storage Stability
270	Substrates and Products (Substrate)
61	Subunits
224	Synonyms
1	Systematic Name
17	Temperature Optimum [°C]
3	Temperature Range [°C]
11	Temperature Stability [°C]
59	Turnover Number [1/s]

Systematic Name

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Systematic Name on EC 1.14.16.1 - phenylalanine 4-monooxygenase

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L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase (4-hydroxylating)

The active centre contains mononuclear iron(II). The reaction involves an arene oxide that rearranges to give the phenolic hydroxy group. This results in the hydrogen at C-4 migrating to C-3 and in part being retained. This process is known as the NIH-shift. The 4a-hydroxytetrahydropteridine formed can dehydrate to 6,7-dihydropteridine, both spontaneously and by the action of EC 4.2.1.96, 4a-hydroxytetrahydrobiopterin dehydratase. The 6,7-dihydropteridine must be enzymically reduced back to tetrahydropteridine, by EC 1.5.1.34, 6,7-dihydropteridine reductase, before it slowly rearranges into the more stable but inactive compound 7,8-dihydropteridine.