PAH antibody

Principal name

PAH antibody

Alternative names for PAH antibody

Phenylalanine Hydroxylase, PH, PKU1, PKU, Phenylalanine-4-hydroxylase, Phenylalanine 4-monooxygenase, Phe-4-monooxygenase

SwissProt ID

P00439 (Human), P04176 (Rat), P16331 (Mouse), P17276 (Drome), P90925 (Caeel), Q2KIH7 (Bovin)

Gene ID

5053 (PAH)

Available reactivities

Hu (Human), Ms (Mouse), Rt (Rat), Bov (Bovine), Can (Canine)

Available hosts

Mouse, Rabbit, Goat

Available applications

ELISA (detection) (E(detection)), Paraffin Sections (P), Western blot / Immunoblot (WB), Enzyme Immunoassay (E)

Background of PAH antibody

The PAH gene encodes the enzyme phenylalanine hydroxylase (PAH), which converts phenylalanine to tyrosine and is the rate-limiting enzyme in phenylalanine catabolism. Mammalian PAH is a soluble, homotetrameric protein which is abundantly expressed in human liver. Deficiency of PAH activity results in the autosomal recessive disorder phenylketonuria (PKU), which is characterized by mental retardation unless a low phenylalanine diet is introduced early in life. The PAH gene, which maps to human chromosome 12q23.2, contains all the genetic information necessary to code for functional PAH, demonstrating that a single gene is involved in the classic disease phenotype. Numerous mutations can impair the PAH gene, which result in decreased enzyme activity and give rise to varying degrees of PKU. Multiple isozymes of PAH have been reported to exist, but these are most likely allelic variants of PAH that produce protein subunits with slightly different charge and electrophoretic migration.

General readings

1. Bonyadi M, Omrani O, Moghanjoghi SM, Shiva S,
Mutations of the phenylalanine hydroxylase gene in Iranian Azeri Turkish patients with phenylketonuria. Genetic testing and molecular biomarkers 2010 Apr 14 (2): 233-5.
PMID: 20187763

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Primary Antibodies

Catalog No. Host Iso. Clone Pres. React. Applications  

PAH antibody

  Goat Anti-Phenylalanine Hydroxylase (PAH) Antibody  
(0.3ug/ml) staining of Human Liver lysate (35ug protein in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence. Goat Aff - Purified Bov, Can, Hu, Ms, Rt E, WB
0.1 mg / €325.00
  OriGene Technologies, Inc.

PAH antibody

  PAH Rabbit Polyclonal Antibody  
Western blot analysis of extracts of various cell lines, using PAH antibody. Rabbit IgG Purified Hu, Ms, Rt
0.1 mg / €325.00
  OriGene Technologies, Inc.

PAH antibody

Immunohistochemistry (IHC) analyzes of PAH antibody (Cat.-No.: AP20792PU-N) in paraffin-embedded human brain tissue. Rabbit Aff - Purified Hu, Ms, Rt P, WB
0.1 mg / €350.00
  Acris Antibodies GmbH

CRISPR

Catalog No. Species Donor Vector  

PAH - human gene knockout kit via CRISPR

PAH - human gene knockout kit via CRISPR Human GFP-Puro
1 kit / €1,200.00
  OriGene Technologies, Inc.

PAH - human gene knockout kit via CRISPR

PAH - human gene knockout kit via CRISPR Human mBFP-Neo
1 kit / €1,290.00
  OriGene Technologies, Inc.

PAH - human gene knockout kit via CRISPR

PAH - human gene knockout kit via CRISPR Human Luciferase-Puro
1 kit / €1,290.00
  OriGene Technologies, Inc.

PAH - human gene knockout kit via CRISPR

PAH - human gene knockout kit via CRISPR Human RFP-BSD
1 kit / €1,290.00
  OriGene Technologies, Inc.

Proteins & Growth Factors

Catalog No. Species Pres. Purity Source  

PAH

  Recombinant protein of human phenylalanine hydroxylase (PAH)  
PAH Human > 80 %
Preparation: Recombint protein was captured through anti-DDK affinity column followed by conventiol chromatography steps.
Purity Detail: > 80% as determined by SDS-PAGE and Coomassie blue staining.
HEK293 cells
20 µg / €680.00
  OriGene Technologies, Inc.

Human Lenti ORF Particles

Catalog No.    

Lenti ORF particles, PAH (mGFP-tagged) - Human phenylalanine hydroxylase (PAH), 200 uL, >10^7 TU/mL

Lenti ORF particles, PAH (mGFP-tagged) - Human phenylalanine hydroxylase (PAH), 200 uL, >10^7 TU/mL  
200 µl / €1,050.00
  OriGene Technologies, Inc.

Lenti ORF particles, PAH (Myc-DDK tagged) - Human phenylalanine hydroxylase (PAH) , 200 uL, >10^7 TU/mL

Lenti ORF particles, PAH (Myc-DDK tagged) - Human phenylalanine hydroxylase (PAH) , 200 uL, >10^7 TU/mL  
200 µl / €940.00
  OriGene Technologies, Inc.

10 Item(s)

per page
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