Phenylalanine-4-hydroxylase; PAH; Phe-4-monooxygenase; PAH

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

Polyclonal

Rabbit Ig

Rabbit

This PAH antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 132-161 amino acids from the Central region of human PAH.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.5 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

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.

Metabolism; Signal Transduction

Western Blot (WB), ELISA (EIA)

WB~~1:1000

Western blot analysis of PAH Antibody (Center) in mouse liver tissue lysates (35ug/lane). PAH (arrow) was detected using the purified Pab.

51862

phenylalanine hydroxylase

phenylalanine-4-hydroxylase

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; Oxidoreductase; Amino Acid Metabolism - phenylalanine, tyrosine and tryptophan biosynthesis

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

Bonyadi, M., et al. Genet Test Mol Biomarkers 14(2):233-235(2010)
Dupuis, J., et al. Nat. Genet. 42(2):105-116(2010)
Santos, L.L., et al. Genet. Mol. Res. 9(1):1-8(2010)

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