Anti-PAH Rabbit Polyclonal Antibody for WB, IF/ICC, ELISA - P00439

Core Product Specifications and Parameters

Biological Background: PAH Function and Localization

• The PAH gene encodes phenylalanine-4-hydroxylase (Phe-4-monooxygenase), a key enzyme in phenylalanine metabolism.
• It catalyzes the hydroxylation of L-phenylalanine to L-tyrosine, the rate-limiting step in phenylalanine catabolism.
• This monooxygenase requires iron as a cofactor and is subject to allosteric regulation.
• Post-translational modifications include phosphorylation, which may modulate enzyme activity.
• Mutations in PAH are associated with phenylketonuria (PKU), an autosomal recessive inborn error of metabolism.
• Relevant keywords from UniProt include: 3D-structure, Allosteric enzyme, Disease variant, Iron, Metal-binding, Monooxygenase, Oxidoreductase, Phenylalanine catabolism, Phenylketonuria, Phosphoprotein.

Experimental Guidance and Technical Tips

• The immunogen corresponds to the N-terminal region (residues 1-290) of human PAH, providing broad coverage of conserved functional domains.
• For Western blot, the predicted molecular weight is ~52 kDa; consider potential shifts due to phosphorylation or other modifications.
• When using IF/ICC, optimize fixation and permeabilization protocols for the target cell type to preserve antigenicity.
• Positive controls such as liver tissue or cell lines expressing PAH are recommended for all applications.
• Cross-reactivity with mouse and rat is supported; validate in the relevant species system to confirm reactivity.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio offers comprehensive IVD raw-material sourcing solutions, including validated antibody pairs, monoclonal/polyclonal antibodies, and bulk ancillary reagents. This anti-PAH polyclonal antibody supports research in amino acid metabolism and phenylketonuria, and CamelBio can facilitate access to associated rare-target reagents and technical support. We are committed to bridging from concept to clinic for diagnostic manufacturers and research laboratories.