Phenylalanine-4-hydroxylase; PAH; 1.14.16.1; Phe-4-monooxygenase; PAH

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

Polyclonal

Rabbit

Immunogen affinity purified

Lyophilized

Add 0.2ml of distilled water will yield a concentration of 500ug/ml. (lot specific)

Store at -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for a longer time. Avoid repeated freezing and thawing.

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 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.

Phenylalanine hydroxylase (PAH) is an enzyme that catalyzes the hydroxylation of the aromatic side-chain of phenylalanine to generate tyrosine. It is one of three members of the biopterin-dependent aromatic amino acid hydroxylases, a class of monooxygenase that uses tetrahydrobiopterin (BH4, a pteridine cofactor) and a non-heme iron for catalysis. Deficiency of this enzyme activity results in the autosomal recessive disorder phenylketonuria.

Signal Transduction; Metabolism; Amino Acids; Metabolism; Pathways and Processes; Metabolic Signaling Pathways; amino acid metabolism

Immunohistochemistry (IHC), Western Blot (WB)

Immunohistochemistry (Paraffin-embedded Section): 0.5-1mug/ml (By Heat)
WB: 0.1-0.5mug/ml

Figure 4. IHC analysis of PAH using anti-PAH antibody (MBS1750480).
PAH was detected in paraffin-embedded section of rat kidney tissue. Heat mediated antigen retrieval was performed in citrate buffer (pH6, epitope retrieval solution) for 20 mins. The tissue section was blocked with 10% goat serum. The tissue section was then incubated with 1ug/ml rabbit anti-PAH Antibody (MBS1750480) overnight at 4 degree C. Biotinylated goat anti-rabbit IgG was used as secondary antibody and incubated for 30 minutes at 37 degree C. The tissue section was developed using Strepavidin-Biotin-Complex (SABC) with DAB as the chromogen.

Figure 2. IHC analysis of PAH using anti-PAH antibody (MBS1750480).
PAH was detected in paraffin-embedded section of human renal cancer tissue. Heat mediated antigen retrieval was performed in citrate buffer (pH6, epitope retrieval solution) for 20 mins. The tissue section was blocked with 10% goat serum. The tissue section was then incubated with 1ug/ml rabbit anti-PAH Antibody (MBS1750480) overnight at 4 degree C. Biotinylated goat anti-rabbit IgG was used as secondary antibody and incubated for 30 minutes at 37 degree C. The tissue section was developed using Strepavidin-Biotin-Complex (SABC) with DAB as the chromogen.

Figure 3. IHC analysis of PAH using anti-PAH antibody (MBS1750480).
PAH was detected in paraffin-embedded section of mouse kidney tissue. Heat mediated antigen retrieval was performed in citrate buffer (pH6, epitope retrieval solution) for 20 mins. The tissue section was blocked with 10% goat serum. The tissue section was then incubated with 1ug/ml rabbit anti-PAH Antibody (MBS1750480) overnight at 4 degree C. Biotinylated goat anti-rabbit IgG was used as secondary antibody and incubated for 30 minutes at 37 degree C. The tissue section was developed using Strepavidin-Biotin-Complex (SABC) with DAB as the chromogen.

Figure 1. Western blot analysis of PAH using anti-PAH antibody (MBS1750480).
Electrophoresis was performed on a 5-20% SDS-PAGE gel at 70V (Stacking gel) / 90V (Resolving gel) for 2-3 hours. The sample well of each lane was loaded with 50ug of sample under reducing conditions.
Lane 1: rat liver tissue lysate,
Lane 2: rat kidney tissue lysate,
Lane 3: mouse liver tissue lysate,
Lane 4: mouse kidney tissue lysate,
Lane 5: human HepG2 whole cell lysate.
After Electrophoresis, proteins were transferred to a Nitrocellulose membrane at 150mA for 50-90 minutes. Blocked the membrane with 5% Non-fat Milk/ TBS for 1.5 hour at RT. The membrane was incubated with rabbit anti-PAH antigen affinity purified polyclonal antibody at 0.5ug/mL overnight at 4 degree C, then washed with TBS-0.1%Tween 3 times with 5 minutes each and probed with a goat anti-rabbit IgG-HRP secondary antibody at a dilution of 1:10000 for 1.5 hour at RT. The signal is developed using an Enhanced Chemiluminescent detection (ECL) kit with Tanon 5200 system. A specific band was detected for PAH at approximately 52KD. The expected band size for PAH is at 52KD.

51862 MW

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]

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