PAH; PH; PH4H_HUMAN; Phe 4 monooxygenase; Phe-4-monooxygenase; Phenylalanine 4 hydroxylase; Phenylalanine hydroxylase; Phenylalanine-4-hydroxylase; PKU; PKU1

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

Polyclonal

IgG

Rabbit

PAH antibody detects endogenous levels of total PAH

The antiserum was purified by peptide affinity chromatography using SulfoLink Coupling Resin.

Liquid
Phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.

1mg/ml (lot specific)

Store at -20 degree C. Stable for 12 months from date of receipt.

Manufactured in an ISO 9001:2015 Certified Laboratory.

Manufactured in a lab with traceable raw materials manufactured on site. Coordinated product portfolio of antibodies, pairs, conjugates, recombinant proteins, and immunoassay materials available, please inquire.

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.

Description: 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.
Function: L-phenylalanine + tetrahydrobiopterin + O2 = L-tyrosine + 4a-hydroxytetrahydrobiopterin.
Subunit Structure: Homodimer and homotetramer.
Post-translational Modifications: Phosphorylation at Ser-16 increases basal activity and facilitates activation by the substrate phenylalanine.
Similarity: Belongs to the biopterin-dependent aromatic amino acid hydroxylase family.

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

WB: 1:500-1:2000
IHC: 1:50-1:200

MBS9607112 at 1/100 staining Human prostate tissue by IHC-P. The sample was formaldehyde fixed and a heat mediated antigen retrieval step in citrate buffer was performed. The sample was then blocked and incubated with the antibody for 1.5 hours at 22 degree C. An HRP conjugated goat anti-rabbit antibody was used as the secondary.

Western blot analysis of Mouse liver tissue lysates, using PAH Antibody. The lane on the left is treated with the antigen-specific peptide.

Observed: 52 kDa
Predicted: 52 kDa

phenylalanine hydroxylase

phenylalanine-4-hydroxylase

Phenylalanine-4-hydroxylase

PAH??[Similar Products]

This gene encodes a member of the biopterin-dependent aromatic amino acid hydroxylase protein family. The encoded phenylalanine hydroxylase enzyme hydroxylates phenylalanine to tyrosine and is the rate-limiting step in phenylalanine catabolism. Deficiency of this enzyme activity results in the autosomal recessive disorder phenylketonuria. [provided by RefSeq, Aug 2017]

Catalyzes the hydroxylation of L-phenylalanine to L-tyrosine.

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