Very Long-Chain Specific Acyl-CoA Dehydrogenase, Mitochondrial (ACADVL) Antibody
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Description
ACADVL Antibody is a Rabbit Polyclonal Antibody against ACADVL.
Documents del producto
Product specifications
| Category | Primary Antibodies |
| Immunogen Target | Target: Very Long-Chain Specific Acyl-CoA Dehydrogenase, Mitochondrial (ACADVL) Immunogen: Recombinant protein corresponding to ACADVL. The exact sequence is proprietary. |
| Host | Rabbit |
| Reactivity | Human, Mouse, Rat |
| Assay Type | Concentration: > 0.2 mg/ml |
| Recommended Dilution | ELISA: 1 µg/ml, WB: 1/500 - 1/2000, IF/ICC: 1/50 - 1/200. Optimal dilutions/concentrations should be determined by the end user. |
| Clonality | Polyclonal |
| Conjugation | Unconjugated |
| Isotype | IgG |
| Observed MW | Calculated MW: 70 kDa Observed MW: 70 kDa |
| Purification | Purified by affinity chromatography. |
| Size 1 | 20 µl |
| Size 2 | 100 µl |
| Size 3 | 2 × 100 µl |
| Form | Liquid |
| Tested Applications | ELISA, WB, IF/ICC |
| Buffer | PBS, pH 7.3, containing 0.02% sodium azide, 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 | P49748 |
| Gene ID | 37 |
| NCBI Accession | NP_000009.1 |
| Alias | ACAD6,LCACD,VLCAD |
| Background | Antibody anti-ACADVL |
| 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
The ACADVL gene encodes the enzyme acyl-CoA dehydrogenase very long chain, which plays a vital role in the initial step of the mitochondrial fatty acid beta-oxidation pathway. Specifically, it is targeted to the inner mitochondrial membrane and is specific to long-chain and very-long-chain fatty acids. Mutations in this gene are associated with very long-chain acyl-coenzyme A dehydrogenase deficiency, leading to reduced myocardial fatty acid beta-oxidation and cardiomyopathy. Alternative splicing generates multiple transcript variants encoding different isoforms of the enzyme
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Very long-chain acyl-CoA dehydrogenase (VLCAD) is one of four flavoproteins which catalyze the initial step of the mitochondrial b-oxidation spiral. It belongs to the acyl-CoA dehydrogenase family and is a homodimer of a 71-kDa polypeptide. The molecular mass of the nondenatured trypsinized VLCAD is 98 kDa, by gel filtration chromatography, indicating that it is a homodimer of the 48 kDa(tryptic digest) polypeptide(PMID:9461620). Defects in ACADVL are the cause of acyl-CoA dehydrogenase very long chain deficiency (ACADVLD). It has 2 isoforms(70 kDa and 68 kDa ) produced by alternative splicing and a transit peptide.
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