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| Cat.No | ACP02429 | Target Name | PAH |
|---|---|---|---|
| Target Synonyms | PAH; PH; PH4H_HUMAN; Phe 4 monooxygenase; Phe-4-monooxygenase; Phenylalanine 4 hydroxylase; Phenylalanine hydroxylase; Phenylalanine-4-hydroxylase; PKU; PKU1 | Form | Liquid or Lyophilized powder |
| Expression System | E.coli | Expression Range | 2-452aa |
| Mol Weight | 67.7kDa | Protein Length | Full Length of Mature Protein |
| Purity | Greater than 90% as determined by SDS-PAGE. | Storage Buffer | 5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0. |
| Target Species | Human | Uniprot ID | P00439 |
|---|
Uniprot Id
P00439
Target Species
Human
Target Name
PAH
Target Full Name
Phenylalanine-4-hydroxylase
Target Function
Catalyzes the hydroxylation of L-phenylalanine to L-tyrosine.
Target Involvement
Phenylketonuria (PKU); Non-phenylketonuria hyperphenylalaninemia (Non-PKU HPA); Hyperphenylalaninemia (HPA)
Target Protein Families
Biopterin-dependent aromatic amino acid hydroxylase family
Target Research Area
Signal Transduction
Target Synonyms
PAH; PH; PH4H_HUMAN; Phe 4 monooxygenase; Phe-4-monooxygenase; Phenylalanine 4 hydroxylase; Phenylalanine hydroxylase; Phenylalanine-4-hydroxylase; PKU; PKU1
Target Background
This gene encodes a member of the biopterin-dependent aromatic amino acid hydroxylase protein family. The encoded phenylalanine hydroxylase enzyme hydroxylates phenylalanine to tyrosine and is the rate-limiting step in phenylalanine catabolism. Deficiency of this enzyme activity results in the autosomal recessive disorder phenylketonuria.
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