Overview
Product nameACADVL protein
Protein descriptionRecombinant full length Human ACADVL (amino acids 41-655) with an N terminal His tag; 636 amino acids including tag, MWt 68.5kDa. (UniProt P49748).
Uniprot accessionP49748
Molecular weight68.500kDa inclusive of tags
Protein length615 amino acids
Expression hostE. coli
Properties
Purity> 90
% by SDS-PAGE
Purification notesab98234 was purified using conventional chromatography techniques.
E.C. Number1.3.99.-
FormLiquid
Storage instructionsStore at +4°C short term (1-2 weeks). Aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Storage bufferPreservative: None
Constituents: 10% Glycerol, 0.1M Sodium chloride, 20mM Tris HCl, 1mM EDTA, 1mM DTT, pH 8.0
Concentration information loading...
SequenceMGSSHHHHHHSSGLVPRGSHMAGGAAQLALDKSDSHPSDA LTRKKPAKAESKSFAVGMFKGQLTTDQVFPYPSVLNEEQT QFLKELVEPVSRFFEEVNDPAKNDALEMVEETTWQGLKEL GAFGLQVPSELGGVGLCNTQYARLVEIVGMHDLGVGITLG AHQSIGFKGILLFGTKAQKEKYLPKLASGETVAAFCLTEP SSGSDAASIRTSAVPSPCGKYYTLNGSKLWISNGGLADIF TVFAKTPVTDPATGAVKEKITAFVVERGFGGITHGPPEKK MGIKASNTAEVFFDGVRVPSENVLGEVGSGFKVAMHILNN GRFGMAAALAGTMRGIIAKAVDHATNRTQFGEKIHNFGLI QEKLARMVMLQYVTESMAYMVSANMDQGATDFQIEAAISK IFGSEAAWKVTDECIQIMGGMGFMKEPGVERVLRDLRIFR IFEGTNDILRLFVALQGCMDKGKELSGLGSALKNPFGNAG LLLGEAGKQLRRRAGLGSGLSLSGLVHPELSRSGELAVRA LEQFATVVEAKLIKHKKGIVNEQFLLQRLADGAIDLYAMV VVLSRASRSLSEGHPTAQHEKMLCDTWCIEAAARIREGMA ALQSDPWQQELYRNFKSISKALVERGGVVTSNPLGF
Research Areas
Applications
Our Abpromise guarantee covers the use of
ab98234
in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
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Application
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Notes |
| SDS-PAGE |
SDS-PAGE: Use at an assay dependent dilution. |
Application notesThis peptide can be used with studies using ab155138.
Protein info
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Alternative names
AcadvlAcyl CoA dehydrogenase very long chainAcyl Coenzyme A dehydrogenase very long chainLCACDmitochondrialVery long chain specific acyl CoA dehydrogenaseVery long chain specific acyl CoA dehydrogenase mitochondrialVery long-chain specific acyl-CoA dehydrogenaseVLCAD
see all
FunctionActive toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can accomodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons.
PathwayLipid metabolism; mitochondrial fatty acid beta-oxidation.
Involvement in diseaseDefects in ACADVL are the cause of acyl-CoA dehydrogenase very long chain deficiency (ACADVLD) [MIM:201475]. ACADVLD is an autosomal recessive disease which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form, with early onset, high mortality, and high incidence of cardiomyopathy; a milder childhood form, with later onset, usually with hypoketotic hypoglycemia as the main presenting feature, low mortality, and rare cardiomyopathy; and an adult form, with isolated skeletal muscle involvement, rhabdomyolysis, and myoglobinuria, usually triggered by exercise or fasting.
Sequence similaritiesBelongs to the acyl-CoA dehydrogenase family.
Cellular localizationMitochondrion inner membrane.
References for ACADVL protein (ab98234)
ab98234
has not yet been referenced specifically in any publications.
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"
SDS-PAGE - ACADVL protein (ab98234)15% SDS-PAGE analysis of 3µg ab98234