L-Aminoadipate-Semialdehyde Dehydrogenase-Phosphopantetheinyl Transferase (AASDHPPT) Antibody
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Description
AASDHPPT Antibody is a Rabbit Polyclonal antibody against AASDHPPT. The protein encoded by this gene is similar to Saccharomyces cerevisiae LYS5, which is required for the activation of the alpha-aminoadipate dehydrogenase in the biosynthetic pathway of lysine. Yeast alpha-aminoadipate dehydrogenase converts alpha-biosynthetic-aminoadipate semialdehyde to alpha-aminoadipate. It has been suggested that defects in the human gene result in pipecolic acidemia.
Documents del producto
Product specifications
| Category | Primary Antibodies |
| Immunogen Target | L-Aminoadipate-Semialdehyde Dehydrogenase-Phosphopantetheinyl Transferase (AASDHPPT) |
| Host | Rabbit |
| Reactivity | Human, Mouse, Rat |
| Recommended Dilution | WB: 1/500 - 1/2000. Optimal dilutions/concentrations should be determined by the end user. |
| Clonality | Polyclonal |
| Conjugation | Unconjugated |
| Isotype | IgG |
| Purification | Purified by affinity chromatography. |
| Size 1 | 60 µl |
| Size 2 | 120 µl |
| Size 3 | 200 µl |
| Form | Liquid |
| Tested Applications | WB |
| Buffer | PBS, pH 7.3, containing 0.01% thiomersal, 50% glycerol. |
| Availability | Shipped within 5-10 working days. |
| Storage | Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles. |
| Dry Ice | No |
| UniProt ID | Q9NRN7 |
| Gene ID | 60496 |
| NCBI Accession | NP_056238.2 |
| Alias | AASD-PPT,ACPS,CGI-80,LYS2,LYS5 |
| Background | Antibody anti-AASDHPPT |
| Status | RUO |
| Note | Concentration: 1 mg/ml - |
Descripción
L-Aminoadipate-Semialdehyde Dehydrogenase-Phosphopantetheinyl Transferase (AASDHPPT) is a bifunctional enzyme involved in lysine degradation and coenzyme A biosynthesis. It catalyzes the oxidation of L-aminoadipate semialdehyde to L-aminoadipate and facilitates the attachment of a 4'-phosphopantetheinyl group to carrier proteins, a key step in activating proteins involved in fatty acid and polyketide synthesis. AASDHPPT is expressed ubiquitously but is particularly active in mitochondria, where lysine degradation contributes to cellular energy metabolism and detoxification. Dysregulation of AASDHPPT is associated with metabolic conditions linked to impaired amino acid catabolism, such as glutaric aciduria type 1. Its dual function also highlights its importance in fatty acid metabolism, and mutations affecting AASDHPPT activity may lead to energy imbalances and oxidative stress. Therapeutic approaches targeting AASDHPPT are being explored for metabolic disorders and mitochondrial dysfunction.
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