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Documentos Principais

AV54486

Sigma-Aldrich

Anti-ACADVL antibody produced in rabbit

affinity isolated antibody

Sinônimo(s):

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

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About This Item

Código UNSPSC:
12352203
NACRES:
NA.41

fonte biológica

rabbit

Nível de qualidade

100

conjugado

unconjugated

forma do anticorpo

affinity isolated antibody

tipo de produto de anticorpo

primary antibodies

clone

polyclonal

Formulário

buffered aqueous solution

peso molecular

64 kDa

reatividade de espécies

horse, dog, rat, pig, bovine, human

concentração

0.5 mg - 1 mg/mL

técnica(s)

immunohistochemistry: suitable
western blot: suitable

nº de adesão NCBI

NP_001029031

nº de adesão UniProt

P49748

Condições de expedição

wet ice

temperatura de armazenamento

−20°C

modificação pós-traducional do alvo

unmodified

Informações sobre genes

human ... ACADVL(37)

Categorias relacionadas

Imunogênio

Synthetic peptide directed towards the N terminal region of human ACADVL

Aplicação

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

Ações bioquímicas/fisiológicas

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.

Sequência

Synthetic peptide located within the following region: RPYAGGAAQESKSFAVGMFKGQLTTDQVFPYPSVLNEEQTQFLKELVEPV

forma física

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

Exoneração de responsabilidade

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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Código de classe de armazenamento

10 - Combustible liquids

Classe de risco de água (WGK)

WGK 3

Ponto de fulgor (°F)

Not applicable

Ponto de fulgor (°C)

Not applicable

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Certificados de análise (COA)

Lot/Batch Number
QC22576

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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
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
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

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