anti- ACADVL antibody
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Description
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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Product specifications
| Category | Primary Antibodies |
| Immunogen Target | acyl-Coenzyme A dehydrogenase, very long chain |
| Host | Rabbit |
| Reactivity | human,mouse,rat |
| Recommended Dilution | WB: 1:200-1:2000; IHC: 1:20-1:200 |
| Clonality | polyclonal |
| Conjugation | Unconjugated |
| Isotype | IgG |
| Observed MW | 48 kDa,68-73 kDa |
| Purity | ≥95% as determined by SDS-PAGE |
| Purification | Immunogen affinity purified |
| Size 1 | 100µg |
| Form | liquid |
| Tested Applications | ELISA, WB, IHC |
| Storage | PBS with 0.02% sodium azide and 50% glycerol pH 7.3,-20℃ for 12 months(Avoid repeated freeze / thaw cycles.) |
| UniProt ID | P49748 |
| Gene ID | 37 |
| Alias | ACAD6,LCACD,VLCAD |
| Background | Antibody anti-ACADVL |
| Status | RUO |
| Note | This product is for research use only. |
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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