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| Reactivity: | Human, Mouse, Rat |
| Applications: | WB, IF/IC, ELISA |
| Host Species: | Rabbit |
| Isotype: | IgG |
| Clonality: | Polyclonal antibody |
| Gene Name: | phenylalanine hydroxylase |
| Gene Symbol: | PAH |
| Synonyms: | PH; PKU; PKU1; PAH |
| Gene ID: | 5053 |
| UniProt ID: | P00439 |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-290 of human PAH (NP_000268.1). |
| Dilution: | WB 1:500-1:1000; IF/IC 1:50-1:200 |
| Purification Method: | Affinity purification |
| Concentration: | 0.76 mg/ml |
| Buffer: | Buffer: PBS with 0.02% sodium azide,50% glycerol,pH7.3. |
| Storage: | Store at -20°C. Avoid freeze/thaw cycles. |
| Documents: | Manual-PAH antibody |
Background
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.
Images
 | Western blot analysis of lysates from HepG2 cells, using PAH Rabbit pAb (A1559) at 1:700 dilution. Secondary antibody: HRP-conjugated Goat anti-Rabbit IgG (H+L) (AS014) at 1:10000 dilution. Lysates/proteins: 25μg per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 30s. |
 | Immunofluorescence analysis of HepG2 cells using PAH Rabbit pAb (A1559) at dilution of 1:50 (40x lens). Secondary antibody: Cy3-conjugated Goat anti-Rabbit IgG (H+L) (AS007) at 1:500 dilution. Blue: DAPI for nuclear staining. |
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