ACADVL Antibody (H-7): sc-376239
- ACADVL Antibody (H-7) is a mouse monoclonal IgG1 κ ACADVL antibody, cited in 9 publications, provided at 200 µg/ml
- specific for an epitope mapping between amino acids 505-541 within an internal region of ACADVL of human origin
- ACADVL Antibody (H-7) is recommended for detection of ACADVL of mouse, rat and human origin by WB, IP, IF, IHC(P) and ELISA
- Anti-ACADVL Antibody (H-7) is available conjugated to agarose for IP; HRP for WB, IHC(P) and ELISA; and to either phycoerythrin or FITC for IF, IHC(P) and FCM
- also available conjugated to Alexa Fluor® 488, Alexa Fluor® 546, Alexa Fluor® 594 or Alexa Fluor® 647 for WB (RGB), IF, IHC(P) and FCM, and for use with RGB fluorescent imaging systems, such as iBright™ FL1000, FluorChem™, Typhoon, Azure and other comparable systems
- also available conjugated to Alexa Fluor® 680 or Alexa Fluor® 790 for WB (NIR), IF and FCM; for use with Near-Infrared (NIR) detection systems, such as LI-COR®Odyssey®, iBright™ FL1000, FluorChem™, Typhoon, Azure and other comparable systems
- m-IgG Fc BP-HRP is the preferred secondary detection reagent for ACADVL Antibody (H-7) for WB and IHC(P) applications. This reagent is now offered in a bundle with ACADVL Antibody (H-7) (see ordering information below).
ACADVL Antibody (H-7) is a mouse monoclonal IgG1 kappa light chain antibody that detects ACADVL protein of mouse, rat, and human origin by western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), immunohistochemistry with paraffin-embedded sections (IHCP), and enzyme-linked immunosorbent assay (ELISA). ACADVL (H-7) antibody is available in both non-conjugated and various conjugated forms, including agarose, horseradish peroxidase (HRP), phycoerythrin (PE), fluorescein isothiocyanate (FITC), and multiple Alexa Fluor® conjugates. ACADVL protein, also known as very long chain acyl-Coenzyme A dehydrogenase (VLCAD), plays a crucial role in lipid metabolism as ACADVL catalyzes the first step of the fatty acid beta-oxidation pathway, specifically targeting long-chain and very long-chain fatty acids. This function is vital for energy production, particularly during periods of fasting or prolonged exercise, as ACADVL helps to mobilize stored fat for energy. Deficiencies in ACADVL gene can lead to very long chain acyl-CoA dehydrogenase deficiency (VLCAD deficiency), an autosomal recessive disorder that disrupts the oxidation of long-chain fatty acids, resulting in severe metabolic complications. VLCAD deficiency manifests in various phenotypes, including a severe childhood form associated with high mortality and cardiomyopathy, a milder childhood form with hypoketotic hypoglycemia, and an adult form characterized by myoglobinuria and rhabdomyolysis. Additionally, alternative splicing of ACADVL gene results in two isoforms, further emphasizing the complexity and importance of ACADVL in metabolic health.
Alexa Fluor® is a trademark of Molecular Probes Inc., OR., USA
LI-COR® and Odyssey® are registered trademarks of LI-COR Biosciences
ACADVL Antibody (H-7) References:
- Molecular heterogeneity in very-long-chain acyl-CoA dehydrogenase deficiency causing pediatric cardiomyopathy and sudden death. | Mathur, A., et al. 1999. Circulation. 99: 1337-43. PMID: 10077518
- Very long-chain acyl-CoA dehydrogenase deficiency presenting as acute hypercapnic respiratory failure. | Tong, MK., et al. 2006. Eur Respir J. 28: 447-50. PMID: 16880373
- Polymorphic ventricular tachycardia and abnormal Ca2+ handling in very-long-chain acyl-CoA dehydrogenase null mice. | Werdich, AA., et al. 2007. Am J Physiol Heart Circ Physiol. 292: H2202-11. PMID: 17209005
- Expression and characterization of mutations in human very long-chain acyl-CoA dehydrogenase using a prokaryotic system. | Goetzman, ES., et al. 2007. Mol Genet Metab. 91: 138-47. PMID: 17374501
- Effects of a fat load and exercise on asymptomatic VLCAD deficiency. | Spiekerkoetter, U. 2007. J Inherit Metab Dis. 30: 405. PMID: 17457695
- Very long chain acyl-CoA dehydrogenase deficiency in a pair of mildly affected monozygotic twin sister in their late fifties. | Zia, A., et al. 2007. J Inherit Metab Dis. 30: 817. PMID: 17514507
- Genetic basis for correction of very-long-chain acyl-coenzyme A dehydrogenase deficiency by bezafibrate in patient fibroblasts: toward a genotype-based therapy. | Gobin-Limballe, S., et al. 2007. Am J Hum Genet. 81: 1133-43. PMID: 17999356
- Loss of heterozygosity of 17p13, with possible involvement of ACADVL and ALOX15B, in the pathogenesis of adrenocortical tumors. | Soon, PS., et al. 2008. Ann Surg. 247: 157-64. PMID: 18156936
- Clear correlation of genotype with disease phenotype in very-long-chain acyl-CoA dehydrogenase deficiency. | Andresen, BS., et al. 1999. Am J Hum Genet. 64: 479-94. PMID: 9973285
Ordering Information
| Product Name | Catalog # | UNIT | Price | Qty | FAVORITES | |
ACADVL Antibody (H-7) | sc-376239 | 200 µg/ml | $316.00 | |||
ACADVL Antibody (H-7): m-IgG Fc BP-HRP Bundle | sc-526014 | 200 µg Ab; 10 µg BP | $354.00 | |||
ACADVL Antibody (H-7) AC | sc-376239 AC | 500 µg/ml, 25% agarose | $416.00 | |||
ACADVL Antibody (H-7) HRP | sc-376239 HRP | 200 µg/ml | $316.00 | |||
ACADVL Antibody (H-7) FITC | sc-376239 FITC | 200 µg/ml | $330.00 | |||
ACADVL Antibody (H-7) PE | sc-376239 PE | 200 µg/ml | $343.00 | |||
ACADVL Antibody (H-7) Alexa Fluor® 488 | sc-376239 AF488 | 200 µg/ml | $357.00 | |||
ACADVL Antibody (H-7) Alexa Fluor® 546 | sc-376239 AF546 | 200 µg/ml | $357.00 | |||
ACADVL Antibody (H-7) Alexa Fluor® 594 | sc-376239 AF594 | 200 µg/ml | $357.00 | |||
ACADVL Antibody (H-7) Alexa Fluor® 647 | sc-376239 AF647 | 200 µg/ml | $357.00 | |||
ACADVL Antibody (H-7) Alexa Fluor® 680 | sc-376239 AF680 | 200 µg/ml | $357.00 | |||
ACADVL Antibody (H-7) Alexa Fluor® 790 | sc-376239 AF790 | 200 µg/ml | $357.00 | |||
ACADVL (H-7) Neutralizing Peptide | sc-376239 P | 100 µg/0.5 ml | $68.00 |