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Merck
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Documenti fondamentali

AV54486

Sigma-Aldrich

Anti-ACADVL antibody produced in rabbit

affinity isolated antibody

Sinonimo/i:

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

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About This Item

Codice UNSPSC:
12352203
NACRES:
NA.41

Origine biologica

rabbit

Livello qualitativo

100

Coniugato

unconjugated

Forma dell’anticorpo

affinity isolated antibody

Tipo di anticorpo

primary antibodies

Clone

polyclonal

Forma fisica

buffered aqueous solution

PM

64 kDa

Reattività contro le specie

horse, dog, rat, pig, bovine, human

Concentrazione

0.5 mg - 1 mg/mL

tecniche

immunohistochemistry: suitable
western blot: suitable

N° accesso NCBI

NP_001029031

N° accesso UniProt

P49748

Condizioni di spedizione

wet ice

Temperatura di conservazione

−20°C

modifica post-traduzionali bersaglio

unmodified

Informazioni sul gene

human ... ACADVL(37)

Categorie correlate

Immunogeno

Synthetic peptide directed towards the N terminal region of human ACADVL

Applicazioni

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

Azioni biochim/fisiol

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.

Sequenza

Synthetic peptide located within the following region: RPYAGGAAQESKSFAVGMFKGQLTTDQVFPYPSVLNEEQTQFLKELVEPV

Stato fisico

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

Esclusione di responsabilità

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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Codice della classe di stoccaggio

10 - Combustible liquids

Classe di pericolosità dell'acqua (WGK)

WGK 3

Punto d’infiammabilità (°F)

Not applicable

Punto d’infiammabilità (°C)

Not applicable

Certificati d'analisi (COA)

Cerca il Certificati d'analisi (COA) digitando il numero di lotto/batch corrispondente. I numeri di lotto o di batch sono stampati sull'etichetta dei prodotti dopo la parola ‘Lotto’ o ‘Batch’.

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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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