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| Reactivity: | Human, Mouse, Rat |
| Applications: | WB, ELISA |
| Host Species: | Rabbit |
| Isotype: | IgG |
| Clonality: | Polyclonal antibody |
| Gene Name: | aldehyde dehydrogenase 18 family member A1 |
| Gene Symbol: | ALDH18A1 |
| Synonyms: | GSAS; P5CS; PYCS; SPG9; ADCL3; SPG9A; SPG9B; ARCL3A; ALDH18A1 |
| Gene ID: | 5832 |
| UniProt ID: | P54886 |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-240 of human ALDH18A1 (NP_002851.2). |
| Dilution: | WB 1:500-1:2000 |
| Purification Method: | Affinity purification |
| Concentration: | 0.22 mg/ml |
| Buffer: | PBS with 0.01% thimerosal, 50% glycerol, pH7.3. |
| Storage: | Store at -20°C. Avoid freeze/thaw cycles. |
| Documents: | Manual-ALDH18A1 antibody |
Background
This gene is a member of the aldehyde dehydrogenase family and encodes a bifunctional ATP- and NADPH-dependent mitochondrial enzyme with both gamma-glutamyl kinase and gamma-glutamyl phosphate reductase activities. The encoded protein catalyzes the reduction of glutamate to delta1-pyrroline-5-carboxylate, a critical step in the de novo biosynthesis of proline, ornithine and arginine. Mutations in this gene lead to hyperammonemia, hypoornithinemia, hypocitrullinemia, hypoargininemia and hypoprolinemia and may be associated with neurodegeneration, cataracts and connective tissue diseases. Alternatively spliced transcript variants, encoding different isoforms, have been described for this gene.
Images
 | Western blot analysis of various lysates using ALDH18A1 Rabbit pAb (A12247) at 1:3000 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: 90s. |
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