PAH, Polyclonal Antibody

PH; PKU; PKU1

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

Polyclonal

IgG

Rabbit

Antigen affinity purification

1mg/mL (lot specific)

Store at -20 degree C (regular) and -80 degree C (long term). Avoid freeze / thaw cycles.

Manufactured in an ISO 9001:2015 Certified Laboratory.

Small volumes of anti-PAH 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.

Western Blot (WB), Immunohistochemistry (IHC)

Western blot analysis of extracts of various cell lines, using PAH antibody.

Immunohistochemistry of paraffin-embedded human esophageal cancer using PAH antibody at dilution of 1:100 (400x lens).

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

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