Very Long-Chain Specific Acyl-CoA Dehydrogenase, Mitochondrial (ACADVL) Antibody

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Description
ACADVL Antibody is a Rabbit Polyclonal against ACADVL.
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Product specifications
Category | Primary Antibodies |
Immunogen Target | Very Long-Chain Specific Acyl-CoA Dehydrogenase, Mitochondrial (ACADVL) |
Host | Rabbit |
Reactivity | Human |
Recommended Dilution | ELISA: 1/1000 - 1/2000, WB: 1/200 - 1/1000, IHC: 1/25 - 1/100. Optimal dilutions/concentrations should be determined by the end user. |
Clonality | Polyclonal |
Conjugation | Unconjugated |
Isotype | IgG |
Purification | Antigen Affinity Chromatography. |
Size 1 | 50 µl |
Size 2 | 100 µl |
Form | Liquid |
Tested Applications | ELISA, WB, IHC |
Buffer | PBS, pH 7.4, containing 0.05% NaN3 and 40% 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 |
Alias | ACAD6,LCACD,VLCAD |
Background | Antibody anti-ACADVL |
Status | RUO |
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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