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Literature summary for 1.14.16.1 extracted from

  • Meisburger, S.P.; Taylor, A.B.; Khan, C.A.; Zhang, S.; Fitzpatrick, P.F.; Ando, N.
    Domain movements upon activation of phenylalanine hydroxylase characterized by crystallography and chromatography-coupled small-angle X-ray scattering (2016), J. Am. Chem. Soc., 138, 6506-6516 .
    View publication on PubMedView publication on EuropePMC

Activating Compound

Activating Compound Comment Organism Structure
L-phenylalanine
-
Rattus norvegicus

Cloned(Commentary)

Cloned (Comment) Organism
expressed in COS-7 cells Homo sapiens

Crystallization (Commentary)

Crystallization (Comment) Organism
sitting drop vapor diffusion method, using 0.1 M Bis-Tris-HCl pH 5.5, 25% (w/v) polyethylene glycol 3350, and 0.2 M magnesium chloride Rattus norvegicus

Protein Variants

Protein Variants Comment Organism
L212P the mutant shows about 20% activity compared to the wild type enzyme Homo sapiens
L249F the mutant shows about 55% activity compared to the wild type enzyme Homo sapiens
L249P the mutant shows about 5% activity compared to the wild type enzyme Homo sapiens
R261P the mutant shows about 14% activity compared to the wild type enzyme Homo sapiens
R270K active-site mutation Rattus norvegicus
R270K the mutant shows about 15% activity compared to the wild type enzyme Homo sapiens
S196Y the mutant shows about 20% activity compared to the wild type enzyme Homo sapiens
T380M the mutant shows about 25% activity compared to the wild type enzyme Homo sapiens
Y204C the mutant shows wild type activity Homo sapiens

Molecular Weight [Da]

Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
210000
-
small-angle X-ray scattering Rattus norvegicus

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
-
?
L-phenylalanine + tetrahydrobiopterin + O2 Rattus norvegicus
-
L-tyrosine + 4a-hydroxytetrahydrobiopterin
-
?

Organism

Organism UniProt Comment Textmining
Homo sapiens P00439
-
-
Rattus norvegicus P04176
-
-

Substrates and Products (Substrate)

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

Subunits

Subunits Comment Organism
homotetramer
-
Rattus norvegicus

Synonyms

Synonyms Comment Organism
PAH
-
Homo sapiens
PheH
-
Rattus norvegicus
phenylalanine hydroxylase
-
Homo sapiens
phenylalanine hydroxylase
-
Rattus norvegicus