Human ACADVL full length protein (ab98234)
- Product nameHuman ACADVL full length protein
- DescriptionRecombinant, His tag
- SourceE. coli
- Amino Acid Sequence
- SequenceMGSSHHHHHHSSGLVPRGSHMAGGAAQLALDKSDSHPSDA LTRKKPAKAESKSFAVGMFKGQLTTDQVFPYPSVLNEEQT QFLKELVEPVSRFFEEVNDPAKNDALEMVEETTWQGLKEL GAFGLQVPSELGGVGLCNTQYARLVEIVGMHDLGVGITLG AHQSIGFKGILLFGTKAQKEKYLPKLASGETVAAFCLTEP SSGSDAASIRTSAVPSPCGKYYTLNGSKLWISNGGLADIF TVFAKTPVTDPATGAVKEKITAFVVERGFGGITHGPPEKK MGIKASNTAEVFFDGVRVPSENVLGEVGSGFKVAMHILNN GRFGMAAALAGTMRGIIAKAVDHATNRTQFGEKIHNFGLI QEKLARMVMLQYVTESMAYMVSANMDQGATDFQIEAAISK IFGSEAAWKVTDECIQIMGGMGFMKEPGVERVLRDLRIFR IFEGTNDILRLFVALQGCMDKGKELSGLGSALKNPFGNAG LLLGEAGKQLRRRAGLGSGLSLSGLVHPELSRSGELAVRA LEQFATVVEAKLIKHKKGIVNEQFLLQRLADGAIDLYAMV VVLSRASRSLSEGHPTAQHEKMLCDTWCIEAAARIREGMA ALQSDPWQQELYRNFKSISKALVERGGVVTSNPLGF
- Molecular weight69 kDa including tags
- Amino acids41 to 655
- TagsHis tag N-Terminus
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.
- Purity> 90
% by SDS-PAGE.
ab98234 was purified using conventional chromatography techniques.
- Concentration information loading...
Preparation and Storage
- Stability and Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Constituents: 10% Glycerol, 0.1M Sodium chloride, 20mM Tris HCl, 1mM EDTA, 1mM DTT, pH 8.0
- ACAD 6
- Acyl CoA dehydrogenase very long chain
- Acyl Coenzyme A dehydrogenase very long chain
- Very long chain specific acyl CoA dehydrogenase
- Very long chain specific acyl CoA dehydrogenase mitochondrial
- Very long-chain specific acyl-CoA dehydrogenase
- 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 Human ACADVL full length protein (ab98234)
ab98234 has not yet been referenced specifically in any publications.