ALDH18A1 (Delta-1-Pyrroline-5-Carboxylate Synthase, Aldehyde Dehydrogenase 18 Family, Member A1), Rabbit
Catalog Number:
USB-475057
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| Article Name: | ALDH18A1 (Delta-1-Pyrroline-5-Carboxylate Synthase, Aldehyde Dehydrogenase 18 Family, Member A1), Rabbit |
| Biozol Catalog Number: | USB-475057 |
| Supplier Catalog Number: | 475057 |
| Alternative Catalog Number: | USB-475057-100 |
| Manufacturer: | US Biological |
| Host: | Rabbit |
| Category: | Antikörper |
| Application: | WB |
| Immunogen: | Synthetic peptide corresponding to the N-terminal region of human ALDH18A1 |
| 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.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. Applications: Suitable for use in Western Blot. Other applications not tested. Recommended Dilution: Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. |
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