Product Name :
PAH monoclonal antibody Background :
The PAH gene encodes the enzyme phenylalanine hydroxylase (PAH), which converts phenylalanine to tyrosine and is the rate-limiting enzyme in phenylalanine catabolism. Mammalian PAH is a soluble, homotetrameric protein which is abundantly expressed in human liver. Deficiency of PAH activity results in the autosomal recessive disorder phenylketonuria (PKU), which is characterized by mental retardation unless a low phenylalanine diet is introduced early in life. The PAH gene, which maps to human chromosome 12q23.2, contains all the genetic information necessary to code for functional PAH, demonstrating that a single gene is involved in the classic disease phenotype. Numerous mutations can impair the PAH gene, which result in decreased enzyme activity and give rise to varying degrees of PKU. Multiple isozymes of PAH have been reported to exist, but these are most likely allelic variants of PAH that produce protein subunits with slightly different charge and electrophoretic migration. Product :
Mouse IgG1 kappa. Liquid in PBS, pH 7.3, 30% glycerol, and 0.01% sodium azide. Storage&Stability :
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles. Specificity :
Recognizes endogenous levels of PAH protein. Immunogen :
KLH-conjugated synthetic peptide encompassing a sequence within the center region of human PAH. The exact sequence is proprietary. Conjugate :
Unconjugated Modification :
Unmodification

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  Western blot analysis of PAH expression in HepG2 (A), mouse liver (B), rat liver (C) whole cell lysates.
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  Immunohistochemical analysis of PAH staining in mouse kidney formalin fixed paraffin embedded tissue section. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH 6.0). The section was then incubated with the antibody at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX.

Bioworld Biotech only provide peptides for our antibodies and do not provide additional peptide customization services.

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Price/Size :

USD 368/1mg/vial

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Tips: 

For phospho antibody, we provide phospho peptide（0.5mg) and non-phospho peptide(0.5mg).

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Describe :

Blocking peptides are peptides that bind specifically to the target antibody and block antibody binding. These peptide usually contains the epitope recognized by the antibody. Antibodies bound to the blocking peptide no longer bind to the epitope on the target protein. This mechanism is useful when non-specific binding is an issue, for example, in Western blotting (WB) and Immunohistochemistry (IHC). By comparing the staining from the blocked antibody versus the antibody alone, one can see which staining is specific; Specific binding will be absent from the western blot or IHC performed with the neutralized antibody.

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Formula:

Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 10 mg/ml.The purity is >90%,tested by HPLC and MS.

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Storage:

The freeze-dried powder is more stable. For short time at 2-8°C. For long term storage store at -20°C. 

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Note :

This product is for research use only (RUO only). Not for use in diagnostic or therapeutic procedures.

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