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Literature summary for 1.14.16.1 extracted from
- A novel approach for enzyme replacement therapy. The use of phenylalanine hydroxylase-based fusion proteins for the treatment of phenylketonuria (2007), J. Biol. Chem., 282, 23402-23409.
Cloned(Commentary)
| Cloned (Comment) | Organism |
|---|---|
| gene pah, expression of His-tagged enzyme in fusion with HIV transactivator of transcription protein in Escherichia coli strain BL21(DE3), subcloning in Escherichi acoli strain DH5alpha | Homo sapiens |
Protein Variants
| Protein Variants | Comment | Organism |
|---|---|---|
| additional information | phenylketonuria results from a mutation in the liver enzyme phenylalanine hydroxylase, the disease is correlated with high and persistent levels of Phe in the plasma plasma of PKU patients causing permanent neurological damage. Construction of PAH-based fusion proteins with delivery moieties based on the HIV-transactivator of transcription peptide, and fragments of human hepatocyte growth factor, i.e. N-terminal and first, second, and third kringle domains, respectively, of HGF, aiming to specifically target PAH to the liver, which retain PAH activity after being internalized into liver cells, effects of the transgenic construct in HuH7, HepG2, and Colo205 cells | Homo sapiens |
Molecular Weight [Da]
| Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
|---|---|---|---|
| 53000 | - |
x * 53000, recombinant enzyme, SDS-PAGE | Homo sapiens |
Natural Substrates/ Products (Substrates)
| Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| phenylalanine + tetrahydrobiopterin + O2 | Homo sapiens | PAH is a key enzyme in the metabolic pathway of phenylalanine. Deficiency in PAH leads to high and persistent levels of this amino acid in theplasma of phenylketonuria patients, causing permanent neurological damage | tyrosine + 4a-hydroxytetrahydrobiopterin | - |
ir |  |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Homo sapiens | - |
- |
- |
Purification (Commentary)
| Purification (Comment) | Organism |
|---|---|
| recombinant His-tagged enzyme in fusion with HIV transactivator of transcription protein from Escherichia coli strain BL21(DE3) by nickel affinity chromatography | Homo sapiens |
Source Tissue
| Source Tissue | Comment | Organism | Textmining |
|---|---|---|---|
| liver | - |
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 | - |
ir | |
| phenylalanine + tetrahydrobiopterin + O2 | PAH is a key enzyme in the metabolic pathway of phenylalanine. Deficiency in PAH leads to high and persistent levels of this amino acid in theplasma of phenylketonuria patients, causing permanent neurological damage | Homo sapiens | tyrosine + 4a-hydroxytetrahydrobiopterin | - |
ir | |
Subunits
| Subunits | Comment | Organism |
|---|---|---|
| ? | x * 53000, recombinant enzyme, SDS-PAGE | Homo sapiens |
Synonyms
| Synonyms | Comment | Organism |
|---|---|---|
| PAH | - |
Homo sapiens |
| phenylalanine hydroxylase | - |
Homo sapiens |
Temperature Optimum [°C]
| Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
|---|---|---|---|
| 30 | - |
assay at | Homo sapiens |
pH Optimum
| pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
|---|---|---|---|
| 7 | - |
assay at | Homo sapiens |
Cofactor
| Cofactor | Comment | Organism | Structure |
|---|---|---|---|
| tetrahydrobiopterin | - |
Homo sapiens | |
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