PAH Antibody (H-2): sc-271258

PAH Antibody (H-2) is a mouse monoclonal IgG1 kappa light chain antibody that detects PAH protein of mouse, rat, and human origin by western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), immunohistochemistry, and enzyme-linked immunosorbent assay (ELISA). PAH Antibody (H-2) is available in both non-conjugated and various conjugated forms, including agarose, horseradish peroxidase (HRP), phycoerythrin (PE), fluorescein isothiocyanate (FITC), and multiple Alexa Fluor® conjugates. The PAH gene encodes phenylalanine hydroxylase, which plays a crucial role in converting phenylalanine to tyrosine, serving as the rate-limiting enzyme in phenylalanine catabolism. This enzymatic function is vital for proper metabolism of amino acids and prevention of toxic phenylalanine accumulation, which can lead to serious neurological issues. PAH protein is primarily located in the liver, where abundant expression highlights this organ′s central role in amino acid metabolism. Deficiency in PAH activity leads to phenylketonuria (PKU), an autosomal recessive disorder characterized by cognitive impairment if not managed with a low-phenylalanine diet from an early age. The PAH gene, located on human chromosome 12q23.2, contains all necessary genetic information to produce functional PAH, illustrating the significance of this single gene in the classic disease phenotype. Various mutations in PAH can result in decreased enzyme activity, leading to different severities of PKU, and while multiple isozymes of PAH have been reported, these are likely allelic variants that produce protein subunits with slightly different charges and electrophoretic migration patterns.

Alexa Fluor^® is a trademark of Molecular Probes Inc., OR., USA

LI-COR^® and Odyssey^® are registered trademarks of LI-COR Biosciences

PAH Antibody (H-2) References:

1. A heteroallelic mutant mouse model: A new orthologue for human hyperphenylalaninemia.  |  Sarkissian, CN., et al. 2000. Mol Genet Metab. 69: 188-94. PMID: 10767173
2. [Clinical, biomedical , neurological and molecular study of 11 patients with new mutations in PAH gene].  |  Mallolas, J., et al. Rev Neurol. 31: 907-10. PMID: 11244681
3. Founder effect of a prevalent phenylketonuria mutation in the Oriental population.  |  Wang, T., et al. 1991. Proc Natl Acad Sci U S A. 88: 2146-50. PMID: 2006152
4. [Immunochemical detection and characteristics of the subunit composition of phenylalanine hydroxylase in the brain of man].  |  Chestkov, VV. and Laptev, AV. 1988. Biull Eksp Biol Med. 106: 30-4. PMID: 3401571
5. Gene transfer and expression of human phenylalanine hydroxylase.  |  Ledley, FD., et al. 1985. Science. 228: 77-9. PMID: 3856322
6. The distributions of subunit numbers and subunit sizes of enzymes: a study of the products of 100 human gene loci.  |  Hopkinson, DA., et al. 1976. Ann Hum Genet. 39: 383-411. PMID: 782337
7. Spectroscopic and kinetic properties of unphosphorylated rat hepatic phenylalanine hydroxylase expressed in Escherichia coli. Comparison of resting and activated states.  |  Kappock, TJ., et al. 1995. J Biol Chem. 270: 30532-44. PMID: 8530485
8. Structure of tetrameric human phenylalanine hydroxylase and its implications for phenylketonuria.  |  Fusetti, F., et al. 1998. J Biol Chem. 273: 16962-7. PMID: 9642259

Monoclonal antibodies for mammalian and non-mammalian protein targets represent essentially all targets covered by polyclonal antibodies. Over 8,300 of our antibodies are offered as ImmunoCruz^® conjugates which eliminates the need for a secondary antibody.

Santa Cruz Biotechnology antibodies have over 360,000 research citations. Anti-PAH Antibody (H-2) has 6 citations in a variety of scientific publications.

Primary antibodies like Anti-PAH Antibody (H-2) for mammalian target proteins are recommended for the detection of a range of mammalian species, primarily of mouse, rat and human species. Many mammalian antibodies are also ideal for veterinary research and reactive with ovine, porcine, caprine, feline, canine, bovine and equine protein targets. Santa Cruz Biotechnology primary monoclonal antibodies are suitable for laboratory research applications and are not intended for diagnostic or therapeutic use.

PAH Antibody (H-2) Specifications

Alternate Product Name: PAH

Product Clone Name: H-2

Classification: Primary Antibody

Clonality: Monoclonal Antibody

Antibody Isotype: IgG₁ kappa light chain

Amino Acids: 404-432

Epitope Target: PAH

Epitope Species: human

Product Reactivity/Detection: PAH

Species Reactivity/Detection: mouse, rat, human

Additional Mammalian Reactivity: equine, canine and porcine

Additional Non-Mammalian Reactivity: N/A

Applications: WB, IP, IF, IHC(P), ELISA

Vialing Information: Each vial contains 200 µg IgG₁ kappa light chain in 1.0 ml of PBS with < 0.1% sodium azide and 0.1% gelatin

Research Citations: 6 citations in various scientific publications

Available Conjugates/Forms for PAH Antibody (H-2): AC: Agarose, AF488: Alexa Fluor^® 488, AF546: Alexa Fluor^® 546, AF594: Alexa Fluor^® 594, AF647: Alexa Fluor^® 647, AF680: Alexa Fluor^® 680, AF790: Alexa Fluor^® 790, FITC: Fluoroscein, HRP: Horseradish Peroxidase, P: Blocking Peptide, PE: Phycoerythrin