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The antibody against PAH was raised in Rabbit using a synthetic peptide corresponding to a sequence within amino acids 1-100 of human PAH (P00439) as the immunogen. The monoclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, IF/ICC, ELISA.
The antibody against PAH was raised in Rabbit using a synthetic peptide corresponding to a sequence within amino acids 1-100 of human PAH (P00439) as the immunogen. The monoclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, IF/ICC, ELISA.
| Cat.No | ADA-13312A | Clonality | Monoclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | PAH |
| Target Synonyms | PH; PKU; PKU1; PAH | Form | Liquid |
| Species Reactivity | Human, Mouse, Rat | Isotype | IgG |
| Storage Buffer | 50% Glycerol, 0.05% BSA, PBS with 0.02% sodium azide, pH7.3. | Purification Method | Affinity purification |
| Positive Samples | Mouse kidney, HepG2, Mouse liver, Rat liver | Application | ELISA, WB, IF/ICC |
| Immunogen Description | A synthetic peptide corresponding to a sequence within amino acids 1-100 of human PAH (P00439). | Target Species | Human |
|---|---|---|---|
| Immunogen Sequence | MSTAVLENPGLGRKLSDFGQETSYIEDNCNQNGAISLIFSLKEEVGALAKVLRLFEENDVNLTHIESRPSRLKKDEYEFFTHLDKRSLPALTNIIKILRH | 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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