anti- ACADVL antibody
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info@markelab.com
Precio
Precio a consultar
Antibody anti-ACADVL
proveedor
FineTestreference
FNab00068Tested Applications
ELISA, WB, IHCreactivity
human,mouse,ratstatus
RUOclonality
polyclonalDescripción
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.
Background
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
Características del producto
category
Primary Antibodies
clonality
polyclonal
reactivity
human,mouse,rat
immunogen target
acyl-Coenzyme A dehydrogenase, very long chain
host
Rabbit
isotype
IgG
conjugation
Unconjugated
form
liquid
tested applications
ELISA, WB, IHC
observerd MW
48 kDa,68-73 kDa
purity
≥95% as determined by SDS-PAGE
purification
Immunogen affinity purified
recommended dilution
WB: 1:200-1:2000; IHC: 1:20-1:200
size 1
100µg
storage
PBS with 0.02% sodium azide and 50% glycerol pH 7.3,-20℃ for 12 months(Avoid repeated freeze / thaw cycles.)
or code
ACADVL
alias
ACAD6,LCACD,VLCAD
uniprot id
note
This product is for research use only.
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