Human Very Long-Chain Specific Acyl-CoA Dehydrogenase, Mitochondrial (ACADVL) Protein
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Description
ACADVL Protein is a recombinant enzyme.
Documents del producto
Product specifications
| Category | Proteins and Peptides |
| Immunogen Target | Very Long-Chain Specific Acyl-CoA Dehydrogenase, Mitochondrial (ACADVL) |
| Host | E. coli |
| Recommended Dilution | Optimal dilutions/concentrations should be determined by the end user. |
| Origin | Human |
| Expression | Recombinant |
| Purity | > 90% (SDS-PAGE) |
| Size 1 | 2 µg |
| Size 2 | 10 µg |
| Size 3 | 1 mg |
| Form | Liquid |
| Tested Applications | SDS-PAGE |
| Availability | Shipped within 5-10 working days. |
| Dry Ice | No |
| UniProt ID | P49748 |
| Alias | ACAD6,LCACD,VLCAD |
| Background | Protein ACADVL |
| Status | RUO |
| Note | This product is for research use only. Not for human consumption, cosmetic, therapeutic or diagnostic use. |
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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ACADVL antibody
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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Recombinant Human ACADVL
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Human Very Long-Chain Specific Acyl-CoA Dehydrogenase, Mitochondrial (ACADVL) Protein
ACADVL Protein is a recombinant enzyme.
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