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Literature summary for 1.14.16.1 extracted from

  • Nascimento, C.; Leandro, J.; Tavares de Almeida, I.; Leandro, P.
    Modulation of the activity of newly synthesized human phenylalanine hydroxylase mutant proteins by low-molecular-weight compounds (2008), Protein J., 27, 392-400.
    View publication on PubMed

Activating Compound

Activating Compound Comment Organism Structure
glycerol does no affect the wild-type enzyme activity at 1-5%, but increases the activity of the mutant enzymes about 1-3fold, overview Homo sapiens

Cloned(Commentary)

Cloned (Comment) Organism
gene pah, expression of His6-tagged wild-type and mutant enzymes in Escherichia coli Homo sapiens

Protein Variants

Protein Variants Comment Organism
I65T site-directed mutagenesis, the mutant shows reduced activity compared to the wild-type enzyme, but the mutant can be rescued by co-expression of chaperones GroEL and GroES Homo sapiens
R261Q site-directed mutagenesis, the mutant shows reduced activity compared to the wild-type enzyme, but the mutant can be rescued by co-expression of chaperones GroEL and GroES Homo sapiens
R270K site-directed mutagenesis, the mutant shows reduced activity compared to the wild-type enzyme, but the mutant can be rescued by co-expression of chaperones GroEL and GroES Homo sapiens
V388M site-directed mutagenesis, the mutant shows reduced activity compared to the wild-type enzyme, but the mutant can be rescued by co-expression of chaperones GroEL and GroES Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
L-phenylalanine + tetrahydrobiopterin + O2 Homo sapiens
-
L-tyrosine + 4a-hydroxytetrahydrobiopterin
-
?

Organism

Organism UniProt Comment Textmining
Homo sapiens P00439
-
-

Purification (Commentary)

Purification (Comment) Organism
recombinant His6-tagged wild-type and mutant enzymes from Escherichia coli by nickel affinity chromatography Homo sapiens

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
L-phenylalanine + tetrahydrobiopterin + O2
-
Homo sapiens L-tyrosine + 4a-hydroxytetrahydrobiopterin
-
?

Subunits

Subunits Comment Organism
oligomer wild-type and mutant enzymes show different oligomeric states, from dimer to hexamer, overview Homo sapiens

Synonyms

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

Temperature Stability [°C]

Temperature Stability Minimum [°C] Temperature Stability Maximum [°C] Comment Organism
additional information
-
thermal inactivation profiles of the purified wild-type enzyme, and mutants I65T, R261Q and V388M in absence or presence of 1% glycerol or trimethylamine N-oxide, overview Homo sapiens

pH Optimum

pH Optimum Minimum pH Optimum Maximum Comment Organism
7.5
-
assay at Homo sapiens

Cofactor

Cofactor Comment Organism Structure
tetrahydrobiopterin
-
Homo sapiens