Very Long-Chain Specific Acyl-CoA Dehydrogenase, Mitochondrial (ACADVL) Antibody

Este producto es parte de ACADVL - Very Long-Chain Specific Acyl-CoA Dehydrogenase MitochondriaL
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260€ (50 µl)

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935106861
info@markelab.com
name
Very Long-Chain Specific Acyl-CoA Dehydrogenase, Mitochondrial (ACADVL) Antibody
category
Primary Antibodies
provider
Abbexa
reference
abx339644
tested applications
ELISA, WB

Description

ACADVL Antibody is a Rabbit Polyclonal against ACADVL.

Documents del producto

Instrucciones
Data sheet
Descargar

Product specifications

Category
Primary Antibodies
Immunogen Target
Very Long-Chain Specific Acyl-CoA Dehydrogenase, Mitochondrial (ACADVL)
Host
Rabbit
Reactivity
Human
Recommended Dilution
ELISA: 1/1000 - 1/2000, WB: 1/200 - 1/1000. Optimal dilutions/concentrations should be determined by the end user.
Clonality
Polyclonal
Conjugation
Unconjugated
Isotype
IgG
Purification
Antigen Affinity Chromatography.
Size 1
50 µl
Size 2
100 µl
Form
Liquid
Tested Applications
ELISA, WB
Buffer
PBS, pH 7.4, containing 0.05% NaN3 and 40% Glycerol.
Availability
Shipped within 5-10 working days.
Storage
Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles.
Dry Ice
No
UniProt ID
P49748
Gene ID
37
Alias
ACAD6,LCACD,VLCAD
Background
Antibody anti-ACADVL
Status
RUO

Descripción

The ACADVL gene encodes the enzyme acyl-CoA dehydrogenase very long chain, which plays a vital role in the initial step of the mitochondrial fatty acid beta-oxidation pathway. Specifically, it is targeted to the inner mitochondrial membrane and is specific to long-chain and very-long-chain fatty acids. Mutations in this gene are associated with very long-chain acyl-coenzyme A dehydrogenase deficiency, leading to reduced myocardial fatty acid beta-oxidation and cardiomyopathy. Alternative splicing generates multiple transcript variants encoding different isoforms of the enzyme

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Very long-chain acyl-CoA dehydrogenase (VLCAD) is one of four flavoproteins which catalyze the initial step of the mitochondrial b-oxidation spiral. It belongs to the acyl-CoA dehydrogenase family and is a homodimer of a 71-kDa polypeptide. The molecular mass of the nondenatured trypsinized VLCAD is 98 kDa, by gel filtration chromatography, indicating that it is a homodimer of the 48 kDa(tryptic digest) polypeptide(PMID:9461620). Defects in ACADVL are the cause of acyl-CoA dehydrogenase very long chain deficiency (ACADVLD). It has 2 isoforms(70 kDa and 68 kDa ) produced by alternative splicing and a transit peptide.

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