PH / PAH, Polyclonal Antibody

Anti-PH / PAH Antibody (Internal) IHC-plus; PAH; Phe-4-monooxygenase; PKU1; PH; Phenylalanine 4-monooxygenase; Phe-4- monooxygenase; Phenylalanine hydroxylase; Phenylalanine-4-hydroxylase; PKU; Human PH; PAH

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Goat

Human PAH / Phenylalanine Hydroxylase.

Immunoaffinity Purified

Tris-buffered saline, pH 7.3, 0.5% BSA, 0.02% sodium azide

0.5 mg/ml (lot specific)

Store at -20 degree C. Minimize freezing and thawing.

Small volumes of anti-PH antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

PAH encodes the enzyme phenylalanine hydroxylase that is the rate-limiting step in phenylalanine catabolism. Deficiency of this enzyme activity results in the autosomal recessive disorder phenylketonuria.

Immunohistochemistry (IHC - Paraffin), Western Blot (WB), ELISA (EIA)

ELISA (1:32000), IHC-P (3.75 ug/ml), WB (0.1 - 0.3 ug/ml)
Usage: Peptide ELISA: antibody detection limit dilution 1:32000. Western blot: Approx 48kD band observed in Human Liver lysates (calculated MW of 51.9kD according to NP_000268.1). Recommended concentration: 0.3-1 ug/ml.

Anti-PH / PAH antibody IHC staining of human kidney. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval.

PAH antibody (0.3 ug/ml) staining of Human Liver lysate (35 ug protein/ml in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence.

51,862 Da

phenylalanine hydroxylase

phenylalanine-4-hydroxylase; phe-4-monooxygenase; phenylalanine 4-monooxygenase

Phenylalanine-4-hydroxylase

PAH  [Similar Products]

PH4H_HUMAN

PAH encodes the enzyme phenylalanine hydroxylase that is the rate-limiting step in phenylalanine catabolism. Deficiency of this enzyme activity results in the autosomal recessive disorder phenylketonuria. [provided by RefSeq, Jul 2008]

PAH: phenylalanine hydroxylase that is the rate-limiting step in phenylalanine catabolism. Belongs to the biopterin-dependent aromatic amino acid hydroxylase family. Deficiency of this enzyme activity results in the autosomal recessive disorder phenylketonuria.

Protein type: EC 1.14.16.1; Amino Acid Metabolism - phenylalanine, tyrosine and tryptophan biosynthesis; Oxidoreductase

Chromosomal Location of Human Ortholog: 12q22-q24.2

Cellular Component: cytosol

Molecular Function: amino acid binding; iron ion binding; phenylalanine 4-monooxygenase activity

Biological Process: L-phenylalanine catabolic process; catecholamine biosynthetic process; amino acid biosynthetic process; neurotransmitter biosynthetic process

Disease: Phenylketonuria

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.