Product Description

PAH Monoclonal Antibody

Sizes: 50μl, 100μl

Catalogue Numbers: MB66750-50, MB66750-100

Lead times: 1-2 weeks, if manufacturer has product in stock

Manufacturer/Ship Location: China

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 resμlts 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 resμlt in decreased enzyme activity and give rise to varying degrees of PKU. Mμltiple 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.

Category: Primary Antibody

Reactivity: Human, Mouse, Rat

Host: Mouse

Applications: WB, IHC

Alternate Names: Phenylalanine-4-hydroxylase, PAH, Phe-4-monooxygenase

Clonality: Monoclonal

Conjugate: Unconjugated

Modification: Unmodification

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

Dilution: WB (1/500 - 1/1000), IHC (1/50 - 1/200)

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

Specificity: Recognizes endogenous levels of PAH protein.

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.

SwissProt: P00439

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

Research Use Only