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

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

Monoclonal

IgG1,kappa

717CT21.1.1

Mouse

This PAH antibody is generated from a mouse immunized with a KLH conjugated synthetic peptide between 127-161 amino acids from the Central region of human PAH.

Purified Mouse Monoclonal Antibody (Mab)

Purified monoclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein G column, followed by dialysis against PBS.

Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°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.

Crown Antibodies; Metabolism; Signal Transduction

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

IHC~~1:25
WB~~1:1000

Immunohistochemical analysis of paraffin-embedded H. liver section using PAH Antibody (Center). MBS9200125 was diluted at 1:25 dilution. A peroxidase-conjugated goat anti-mouse IgG at 1:400 dilution was used as the secondary antibody, followed by DAB staining.

MBS9200125 staining PAH in human kidney tissue sections by Immunohistochemistry (IHC-P -paraformaldehyde-fixed, paraffin-embedded sections). Tissue was fixed with formaldehyde and blocked with 3% BSA for 0. 5 hour at room temperature; antigen retrieval was by heat mediation with a citrate buffer (pH6). Samples were incubated with primary antibody (1/25) for 1 hours at 37°C. A undiluted biotinylated goat polyvalent antibody was used as the secondary antibody.

Immunohistochemical analysis of paraffin-embedded M. kidney section using PAH Antibody (Center). MBS9200125 was diluted at 1:25 dilution. A peroxidase-conjugated goat anti-rabbit IgG at 1:400 dilution was used as the secondary antibody, followed by DAB staining.

Western blot analysis of lysates from HepG2 cell line and mouse liver rat liver tissue lysates (from left to right) using PAH Antibody (Center). MBS9200125 was diluted at 1:1000 at each lane. A goat anti-mouse IgG H&L(HRP) at 1:3000 dilution was used as the secondary antibody. Lysates at 35ug per lane.

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

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

Kwok S.C.M.,et al.Biochemistry 24:556-561(1985).
Scriver C.R.,et al.Submitted (SEP-1997) to the EMBL/GenBank/DDBJ databases.
Cotton R.G.,et al.Biochem. J. 255:193-196(1988).
Miranda F.F.,et al.J. Biol. Chem. 277:40937-40943(2002).
Siltberg-Liberles J.,et al.Gene 427:86-92(2008).

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