Acyl-CoA Dehydrogenase Family Member 8 (ACAD8) Antibody (HRP)
169€ (20 µg)
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Name
Acyl-CoA Dehydrogenase Family Member 8 (ACAD8) Antibody (HRP)
Category
Primary Antibodies
Provider
Abbexa
Reference
abx312312
Tested Applications
ELISA
Description
ACAD8 Antibody (HRP) is a Rabbit Polyclonal against ACAD8 conjugated to HRP.
Documentos del producto
Instrucciones
Data sheet
Especificaciones del producto
| Category | Primary Antibodies |
| Immunogen Target | Target: Acyl-CoA Dehydrogenase Family Member 8 (ACAD8) Immunogen: Recombinant human Isobutyryl-CoA dehydrogenase, mitochondrial protein (201-415AA). |
| Host | Rabbit |
| Reactivity | Human |
| Recommended Dilution | Optimal dilutions/concentrations should be determined by the end user. |
| Clonality | Polyclonal |
| Conjugation | HRP |
| Isotype | IgG |
| Purity | > 95% |
| Purification | Purified by Protein G. |
| 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 exposure to light. Avoid repeated freeze/thaw cycles. |
| Dry Ice | No |
| UniProt ID | Q9UKU7 |
| Gene ID | 27034 |
| NCBI Accession | NP_055199.1, NM_014384.2 |
| OMIM | 604773 |
| Alias | ACAD-8,ARC42,IBDH |
| Background | Antibody anti-ACAD8 |
| 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. |
Background
ACAD8 is a mitochondrial enzyme involved in the catabolism of isoleucine, a branched-chain amino acid, through its role in the propionyl-CoA pathway. It catalyzes the dehydrogenation of isobutyryl-CoA, an essential step in the breakdown of isoleucine into intermediates that can enter the tricarboxylic acid (TCA) cycle for energy production. ACAD8 is expressed predominantly in tissues with high metabolic activity, including the liver and muscles, where branched-chain amino acid metabolism is critical for energy balance. Mutations in the ACAD8 gene are associated with isobutyryl-CoA dehydrogenase deficiency, a rare metabolic disorder characterized by elevated levels of isobutyryl-carnitine and potential metabolic acidosis. Its role in amino acid metabolism underscores its importance in both energy production and metabolic regulation, particularly during periods of increased energy demand, such as fasting or physical exertion.