Technical Data
A0193-30C
ACADVL (Very Long-chain Specific Acyl-CoA Dehydrogenase, Mitochondrial, VLCAD, VLCAD)
Description:
ACADVL is targeted to the inner mitochondrial membrane where it catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. This acyl-Coenzyme A dehydrogenase is specific to long-chain and very-long-chain fatty acids. A deficiency in its gene product reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy.

Applications:
Suitable for use in ELISA and Western Blot. Other applications not tested.

Recommended Dilution:
ELISA: 1:2000
Western Blot: 1-3ug/ml, human heart lysates observed on ~68kD bands
Optimal dilutions to be determined by the researcher.

Storage and Stability:
May be stored at 4C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
TypeIsotypeCloneGrade
PabAffinity Purified
SizeStorageShippingSourceHost
100ug-20CBlue IceHumanGoat
Concentration:
~0.5mg/ml
Immunogen:
Synthetic peptide corresponding to DKSDSHPSDALTRK-C, from human VLCAD, at N-terminal (near) of the protein (NP_000009.1, NP_001029031.1).
Purity:
Purified by immunoaffinity chromatography.
Form
Supplied as a liquid in Tris saline, 0.02% sodium azide, pH 7.3, 0.5% BSA.
Specificity:
Recognizes human VLCAD.
Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.
1. Gobin-Limballe S, Djouadi F, Aubey F, Olpin S, Andresen BS, Yamaguchi S, Mandel H, Fukao T, Ruiter JP, Wanders RJ, McAndrew R, Kim JJ, Bastin J. Genetic basis for correction of very-long-chain acyl-coenzyme A dehydrogenase deficiency by bezafibrate in patient fibroblasts: toward a genotype-based therapy. Am J Hum Genet. 2007 Dec;81(6):1133-43.