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Merck

AV54486

Sigma-Aldrich

Anti-ACADVL antibody produced in rabbit

affinity isolated antibody

Synonym(e):

Anti-ACAD6, Anti-Acyl-coenzyme A dehydrogenase, very long chain, Anti-LCACD, Anti-VLCAD

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About This Item

UNSPSC-Code:
12352203
NACRES:
NA.41

Biologische Quelle

rabbit

Qualitätsniveau

Konjugat

unconjugated

Antikörperform

affinity isolated antibody

Antikörper-Produkttyp

primary antibodies

Klon

polyclonal

Form

buffered aqueous solution

Mol-Gew.

64 kDa

Speziesreaktivität

horse, dog, rat, pig, bovine, human

Konzentration

0.5 mg - 1 mg/mL

Methode(n)

immunohistochemistry: suitable
western blot: suitable

NCBI-Hinterlegungsnummer

UniProt-Hinterlegungsnummer

Versandbedingung

wet ice

Lagertemp.

−20°C

Posttranslationale Modifikation Target

unmodified

Angaben zum Gen

human ... ACADVL(37)

Immunogen

Synthetic peptide directed towards the N terminal region of human ACADVL

Anwendung

Anti-ACADVL antibody produced in rabbit is suitable for western blotting at a concentration of 1μg/mL.

Biochem./physiol. Wirkung

The protein encoded by ACADVL (Acyl-coenzyme A dehydrogenase, very long chain) gene belongs to acyl-CoA dehydrogenases (ACADs) family and is specific to esters of long-chain and very long chain fatty acids like-palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. It is a homodimer of 67kDa subunits located in mitochondrion inner membrane, where it catalyzes the initial, rate-limiting step of mitochondrial fatty acid β-oxidation. Missense mutations in ACADVL gene may leads to partial carnitine palmitoyltransferase II deficiency. Deficiency of the encoded protein reduces myocardial fatty acid β-oxidation and results in hypertrophic cardiomyopathy.

Sequenz

Synthetic peptide located within the following region: RPYAGGAAQESKSFAVGMFKGQLTTDQVFPYPSVLNEEQTQFLKELVEPV

Physikalische Form

Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

Haftungsausschluss

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.

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Lagerklassenschlüssel

10 - Combustible liquids

WGK

WGK 3

Flammpunkt (°F)

Not applicable

Flammpunkt (°C)

Not applicable


Analysenzertifikate (COA)

Suchen Sie nach Analysenzertifikate (COA), indem Sie die Lot-/Chargennummer des Produkts eingeben. Lot- und Chargennummern sind auf dem Produktetikett hinter den Wörtern ‘Lot’ oder ‘Batch’ (Lot oder Charge) zu finden.

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Die Dokumentenbibliothek aufrufen

Sara Tucci et al.
Biochimica et biophysica acta, 1842(5), 677-685 (2014-02-18)
Hypertrophic cardiomyopathy is a typical manifestation of very long-chain acyl-CoA dehydrogenase deficiency (VLCADD), the most common long-chain β-oxidation defects in humans; however in some patients cardiac function is fully compensated. Cardiomyopathy may also be reversed by supplementation of medium-chain triglycerides
Paul J Isackson et al.
Muscle & nerve, 47(2), 224-229 (2012-11-22)
Twenty-six patients with clinical symptoms of adult onset carnitine palmitoyltransferase II (CPTII) deficiency were examined. All patients had skeletal muscle CPTII enzyme activity levels indicative of heterozygosity for CPT2 mutations, however sequence analysis identified no pathogenic mutations within the CPT2
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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