Delta-1-Pyrroline-5-Carboxylate Synthase (ALDH18A) Antibody
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Description
Rabbit Polyclonal against the ALDH18A protein.
Documents del producto
Product specifications
| Category | Primary Antibodies |
| Immunogen Target | Target: Delta-1-Pyrroline-5-Carboxylate Synthase (ALDH18A) Immunogen: Recombinant fragment corresponding to 10-257 AA of human ALDH18A. |
| Host | Rabbit |
| Reactivity | Human |
| Assay Type | Concentration: Lyophilized form: Not applicable. After reconstitution: 1 mg/ml. |
| Recommended Dilution | ELISA: 1/20000 - 1/80000, WB: 1/500 - 1/2000. Optimal dilutions/concentrations should be determined by the end user. |
| Clonality | Polyclonal |
| Conjugation | Unconjugated |
| Isotype | IgG |
| Observed MW | Observed MW: 87 kDa |
| Purification | Purified by antigen affinity column chromatography. |
| Size 1 | 100 µg |
| Size 2 | 1 mg |
| Form | Lyophilized |
| Tested Applications | ELISA, WB |
| Buffer | Prior to lyophilization: 1% BSA and 0.02% NaN3. |
| Availability | Shipped within 7-15 working days. |
| Storage | Store at -20 °C. Avoid repeated freeze/thaw cycles. |
| Dry Ice | No |
| Alias | P5CS,Aldehyde dehydrogenase family 18 member A1 |
| Background | Antibody anti-ALDH18A1 |
| Status | RUO |
| Note | THIS PRODUCT IS FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC, THERAPEUTIC OR COSMETIC PROCEDURES. NOT FOR HUMAN OR ANIMAL CONSUMPTION. |
Descripción
ALDH18A1, also known as P5CS (pyrroline-5-carboxylate synthase), is a mitochondrial enzyme that catalyzes the conversion of glutamate into pyrroline-5-carboxylate (P5C), a key step in proline and ornithine biosynthesis. This enzyme is crucial for amino acid metabolism, cellular redox balance, and collagen synthesis. ALDH18A1 is highly expressed in tissues with high metabolic activity, such as the liver, kidney, and brain. Mutations in ALDH18A1 are linked to autosomal dominant and recessive forms of P5CS deficiency, which result in hyperammonemia, intellectual disability, and connective tissue disorders due to impaired proline biosynthesis. ALDH18A1 dysfunction also contributes to neurodegenerative diseases and metabolic syndromes by disrupting amino acid metabolism and cellular stress responses. Its role in collagen synthesis makes ALDH18A1 essential for proper skin, bone, and connective tissue maintenance, and it is actively studied for its involvement in aging and fibrosis.
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ALDH18A1 Antibody is a Rabbit Polyclonal antibody against 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. Alternatively spliced transcript variants, encoding different isoforms, have been described for this gene.
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