PAH Antibody (E-8): sc-271257

PAH Antibody (E-8) is a mouse monoclonal IgG2a antibody that detects phenylalanine hydroxylase (PAH) in mouse, rat, and human samples through applications such as western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). PAH is a crucial enzyme that catalyzes the conversion of phenylalanine to tyrosine, serving as the rate-limiting step in phenylalanine catabolism, which is vital for the synthesis of neurotransmitters and other important biomolecules. The proper functioning of PAH is essential, as deficiencies in this enzyme lead to phenylketonuria (PKU), a genetic disorder that can result in severe cognitive impairment if not managed with a strict low-phenylalanine diet from an early age. Located predominantly in the liver, PAH exists as a soluble homotetrameric protein, and PAH structure is critical for enzymatic activity. The PAH gene, situated on human chromosome 12q23.2, encodes all necessary information for producing functional PAH, highlighting the significance of this single gene in the classic PKU phenotype. Various mutations in the PAH gene can lead to reduced enzyme activity, resulting in a spectrum of PKU severity, and while multiple isozymes of PAH have been identified, these variants exhibit slight differences in charge and electrophoretic behavior. Anti-PAH antibody (E-8) is an invaluable tool for researchers studying the implications of PAH in metabolic disorders and exploring potential therapeutic interventions.

The preferred PAH antibody is PAH Antibody (H-2): sc-271258.