AV54486
Anti-ACADVL antibody produced in rabbit
affinity isolated antibody
别名:
Anti-ACAD6, Anti-Acyl-coenzyme A dehydrogenase, very long chain, Anti-LCACD, Anti-VLCAD
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关于此项目
NACRES:
NA.41
UNSPSC Code:
12352203
Conjugate:
unconjugated
Clone:
polyclonal
Application:
IHC, WB
Citations:
3
biological source
rabbit
conjugate
unconjugated
antibody form
affinity isolated antibody
antibody product type
primary antibodies
clone
polyclonal
form
buffered aqueous solution
mol wt
64 kDa
species reactivity
horse, dog, rat, pig, bovine, human
concentration
0.5 mg - 1 mg/mL
technique(s)
immunohistochemistry: suitable, western blot: suitable
NCBI accession no.
UniProt accession no.
shipped in
wet ice
storage temp.
−20°C
target post-translational modification
unmodified
Gene Information
human ... ACADVL(37)
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Immunogen
Synthetic peptide directed towards the N terminal region of human ACADVL
Application
Anti-ACADVL antibody produced in rabbit is suitable for western blotting at a concentration of 1μg/mL.
Biochem/physiol Actions
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.
Physical form
Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
Other Notes
Synthetic peptide located within the following region: RPYAGGAAQESKSFAVGMFKGQLTTDQVFPYPSVLNEEQTQFLKELVEPV
Disclaimer
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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存储类别
10 - Combustible liquids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
法规信息
常规特殊物品
此项目有
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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