HPA019006

Sigma-Aldrich

Anti-ACADVL antibody produced in rabbit

enhanced validation

Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution, Ab1

Synonym(s):
Anti-VLCAD, Anti-Very long-chain specific acyl-CoA dehydrogenase, mitochondrial
Human Protein Atlas Number:

biological source

rabbit

Quality Level

antibody form

affinity isolated antibody

antibody product type

primary antibodies

clone

polyclonal

product line

Prestige Antibodies® Powered by Atlas Antibodies

form

buffered aqueous glycerol solution

species reactivity

mouse, rat, human

packaging

antibody small pack of 25 μL

enhanced validation

orthogonal RNAseq
independent
Learn more about Antibody Enhanced Validation

application(s)

immunoblotting: 0.04-0.4 μg/mL
immunofluorescence: 0.25-2 μg/mL
immunohistochemistry: 1:50-1:200

immunogen sequence

KLIKHKKGIVNEQFLLQRLADGAIDLYAMVVVLSRASRSLSEGHPTAQHEKMLCDTWCIEAAARIREGMAALQSDPWQQELYRNFKSISKALVERGGVVTSNPLGF

conjugate

unconjugated

UniProt accession no.

shipped in

wet ice

storage temp.

−20°C

Gene Information

human ... ACADVL(37)

General description

The gene ACADVL (acyl-CoA dehydrogenase, very long chain) is mapped to human chromosome 17p13.1. It belongs to family of acyl-CoA dehydrogenases (ACADs). The protein exists as a homodimer and associates with the mitochondrial membrane. ACADVL is commonly referred as VLCA (very long-chain specific acyl-CoA dehydrogenase).

Immunogen

Very long-chain specific acyl-CoA dehydrogenase, mitochondrial Precursor recombinant protein epitope signature tag (PrEST)

Biochem/physiol Actions

ACADVL (acyl-CoA dehydrogenase, very long chain) is involved in the initial step of mitochondrial fatty acid β-oxidation. Mutation or absence of ACADVL causes disturbance in mitochondrial β-oxidation, characterized by hepatocellular dysfunction, lipid storage in tissues and cardiac failure. ACADVL deficiency is associated with infantile cardiomyopathy.

Features and Benefits

Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.

Every Prestige Antibody is tested in the following ways:
  • IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
  • Protein array of 364 human recombinant protein fragments.

Linkage

Corresponding Antigen APREST74678.

Physical form

Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide

Legal Information

Prestige Antibodies is a registered trademark of Sigma-Aldrich Co. LLC

Disclaimer

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

RIDADR

NONH for all modes of transport

WGK Germany

WGK 1

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Certificate of Analysis

Certificate of Origin

M Souri et al.
American journal of human genetics, 58(1), 97-106 (1996-01-01)
Very-long-chain acyl-coenzyme A dehydrogenase (VLCAD) deficiency is a newly identified disease. A 105-bp deletion in the VLCAD cDNA in two patients has been reported, and detailed molecular characterization of this disease has remained to be done. We report here five...
H Mendez-Figueroa et al.
Journal of perinatology : official journal of the California Perinatal Association, 30(8), 558-562 (2010-07-30)
Very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency is an enzymatic defect of the fatty acid (FA) beta oxidation pathway. In catabolic states, such as labor and early postpartum period, patients are potentially prone to metabolic decompensation and subsequent rhabdomyolysis with increased...
A Mathur et al.
Circulation, 99(10), 1337-1343 (1999-03-17)
Genetic defects are being increasingly recognized in the etiology of primary cardiomyopathy (CM). Very-long-chain acyl-CoA dehydrogenase (VLCAD) catalyzes the first step in the beta-oxidation spiral of fatty acid metabolism, the crucial pathway for cardiac energy production. We studied 37 patients...
Ryan P McAndrew et al.
The Journal of biological chemistry, 283(14), 9435-9443 (2008-01-30)
Very-long-chain acyl-CoA dehydrogenase (VLCAD) is a member of the family of acyl-CoA dehydrogenases (ACADs). Unlike the other ACADs, which are soluble homotetramers, VLCAD is a homodimer associated with the mitochondrial membrane. VLCAD also possesses an additional 180 residues in the...

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