PAH Monoclonal Antibody-MB66750

PAH Monoclonal Antibody

Product: Mouse IgG1 kappa. Liquid in PBS, pH 7.3, 30% glycerol, and 0.01% sodium azide.

Catalogue Numbers: MB66750-50, MB66750-100

Sizes: 50µl, 100µl

Swiss-Prot: P00439

Host: Mouse

Reactivity: Human, Mouse, Rat

Applications: WB, IHC

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.

Purification and Purity: This antibody is purified through a protein G column.

Specificity: Recognizes endogenous levels of PAH protein.

Note: For research use only, not for use in diagnostic procedure.

Extra Notes: Western blot analysis of PAH expression in HepG2 (A), mouse liver (B), rat liver (C) whole cell lysates., 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 Molecular Weight: ~ 52 kDa

Storage and Stability: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

Alternative Name: Phenylalanine-4-hydroxylase; PAH; Phe-4-monooxygenase

Immunogen: KLH-conjugated synthetic peptide encompassing a sequence within the center region of human PAH. The exact sequence is proprietary.

Conjugate: Unconjugated

Modification: Unmodified