Antibody data
- Antibody Data
- Antigen structure
- References [0]
- Comments [0]
- Validations
- Western blot [1]
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- Product number
- BS3704 - Provider product page
- Provider
- Bioworld Technology, Inc
- Proper citation
- Bioworld Technology Cat#BS3704, RRID:AB_1663193
- Product name
- PAH (R400) polyclonal antibody
- Antibody type
- Polyclonal
- Antigen
- Synthetic peptide, corresponding to amino acids 371-420 of Human PAH.
- Description
- 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.
- Reactivity
- Human, Mouse, Rat
- Host
- Rabbit
- Isotype
- IgG
- Vial size
- 100ul
- Concentration
- 1 mg/ml
- Storage
- Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
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Supportive validation
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- Bioworld Technology, Inc (provider)
- Main image
- Experimental details
- Western blot (WB) analysis of PAH (R400) pAb at 1:500 dilutionLane1:HepG2 whole cell lysateLane2:NIH-3T3 whole cell lysateLane3:H9C2 whole cell lysate