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Key Specifications Table
| Species Reactivity | Key Applications | Host | Format | Antibody Type |
|---|---|---|---|---|
| H | WB, IHC | M | Purified | Monoclonal Antibody |
| Description | |
|---|---|
| Catalogue Number | MABN754 |
| Description | Anti-PAH Antibody, clone 6H10.1 |
| Alternate Names |
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| Background Information | PAH, also known as Phenylalanine-4-hydroxylase , Phe-4-monooxygenase, and encoded by the gene name PAH, belongs to the biopterin-dependent aromatic amino acid hydroxylase family. Phenylalanine hydroxylase is the rate-limiting enzyme of the metabolic pathway that degrades excess phenylalanine. Phenylalanine hydroxylase (PheOH, alternatively PheH or PAH) is an enzyme that catalyzes the hydroxylation of the aromatic side-chain of phenylalanine to generate tyrosine. PheOH is one of three members of the pterin-dependent amino acid hydroxylases, a class of monooxygenase that uses tetrahydrobiopterin (BH4, a pteridine cofactor) and a non-heme iron for catalysis. During the reaction, molecular oxygen is heterolytically cleaved with sequential incorporation of one oxygen atom into BH4 and phenylalanine substrate. PAH has been associated with Phenylketonuria PKU, an autosomal recessive inborn error of phenylalanine metabolism, due to severe phenylalanine hydroxylase deficiency. Additioanlly, PAH has been associated with Non-phenylketonuria hyperphenylalaninemia (Non-PKU HPA), a mild form of phenylalanine hydroxylase deficiency characterized by phenylalanine levels persistently below 600 mumol, which allows normal intellectual and behavioral development without treatment. Finally, PAH may play a role in the Hyperphenylalaninemia (HPA), a mildest form of phenylalanine hydroxylase deficiency. PAH is broadly expressed, with greatest levels in skeletal muscle followed by heart, brain, pancreas and testis. |
| Product Information | |
|---|---|
| Format | Purified |
| Presentation | Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mm NaCl with 0.05% sodium azide. |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | This Anti-PAH antibody is validated for use in WB, IH for the detection of PAH. |
| Key Applications |
|
| Application Notes | Western Blotting Analysis: 1.0 µg/mL from a representative lot detected PAH in 10 µg of human liver tissue lysate. Immunohistochemistry Analysis: A 1:50-250 dilution from a representative lot detected PAH in human cerebral cortex and human liver tissue. |
| Biological Information | |
|---|---|
| Immunogen | GST-tagged recombinant protein corresponding to human PAH. |
| Clone | 6H10.1 |
| Concentration | Please refer to lot specific datasheet. |
| Host | Mouse |
| Isotype | IgG1κ |
| Species Reactivity |
|
| Antibody Type | Monoclonal Antibody |
| Entrez Gene Number |
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| Gene Symbol |
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| Purification Method | Protein g Purified |
| UniProt Number |
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| Molecular Weight | ~52 kDa observed |
| Product Usage Statements | |
|---|---|
| Quality Assurance | Evaluated by Western Blotting in HepG2 cell lysate. Western Blotting Analysis: 1.0 µg/mL of this antibody detected PAH in 10 µg of HepG2 cell lysate. |
| Usage Statement |
|
| Storage and Shipping Information | |
|---|---|
| Storage Conditions | Stable for 1 year at 2-8°C from date of receipt. |
| Packaging Information | |
|---|---|
| Material Size | 100 µg |