Alpha-aminoadipic semialdehyde synthase, mitochondrial (AASS) Antibody (Biotin)
169€ (20 µg)
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935106861
info@markelab.com
name
Alpha-aminoadipic semialdehyde synthase, mitochondrial (AASS) Antibody (Biotin)
category
Primary Antibodies
provider
Abbexa
reference
abx349270
tested applications
ELISA
Description
Alpha-aminoadipic semialdehyde synthase, mitochondrial (AASS) Antibody (Biotin) is a Rabbit Polyclonal antibody conjugated to Biotin for the detection of Human AASS.
Documents del producto
Instrucciones
Data sheet
Product specifications
| Category | Primary Antibodies |
| Immunogen Target | Alpha-aminoadipic semialdehyde synthase, mitochondrial (AASS) |
| Host | Rabbit |
| Reactivity | Human |
| Recommended Dilution | Optimal dilutions/concentrations should be determined by the end user. |
| Clonality | Polyclonal |
| Conjugation | Biotin |
| Isotype | IgG |
| Purity | > 95% |
| Purification | Purified by Protein G chromatography. |
| Size 1 | 20 µg |
| Size 2 | 50 µg |
| Size 3 | 100 µg |
| Size 4 | 200 µg |
| Size 5 | 1 mg |
| Form | Liquid |
| Tested Applications | ELISA |
| Buffer | 0.01 M PBS, pH 7.4, 0.03% Proclin-300 and 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 | Q9UDR5 |
| Gene ID | 10157 |
| NCBI Accession | NM_005763 |
| OMIM | 238700 |
| Alias | LKRSDH,LORSDH,LKR/SDH |
| Background | Antibody anti-AASS |
| Status | RUO |
Descripción
Alpha-aminoadipic semialdehyde synthase (AASS) is a bifunctional mitochondrial enzyme involved in the lysine degradation pathway. It catalyzes two sequential reactions: the oxidation of lysine to saccharopine via its lysine-ketoglutarate reductase activity and the subsequent conversion of saccharopine to alpha-aminoadipic semialdehyde via saccharopine dehydrogenase activity. This pathway plays a crucial role in amino acid catabolism and the production of acetyl-CoA, linking it to energy metabolism and the tricarboxylic acid (TCA) cycle. AASS is essential for maintaining lysine homeostasis and energy production, particularly under conditions of low glucose availability. Mutations in the AASS gene can lead to hyperlysinemia, a metabolic disorder characterized by elevated lysine levels in the blood and associated neurological symptoms. Emerging studies suggest AASS may also play a regulatory role in mitochondrial function and oxidative stress responses.
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