Human ACADL full length protein (ab113579)
- Product nameHuman ACADL full length proteinSee all ACADL proteins and peptides ...
- DescriptionRecombinant, His tag
- SourceE. coli
- Amino Acid Sequence
- SequenceMGSSHHHHHHSSGLVPRGSHMGGEERLETPSAKKLTDIGI RRIFSPEHDIFRKSVRKFFQEEVIPHHSEWEKAGEVSREV WEKAGKQGLLGVNIAEHLGGIGGDLYSAAIVWEEQAYSNC SGPGFSIHSGIVMSYITNHGSEEQIKHFIPQMTAGKCIGA IAMTEPGAGSDLQGIKTNAKKDGSDWILNGSKVFISNGSL SDVVIVVAVTNHEAPSPAHGISLFLVENGMKGFIKGRKLH KMGLKAQDTAELFFEDIRLPASALLGEENKGFYYIMKELP QERLLIADVAISASEFMFEETRNYVKQRKAFGKTVAHLQT VQHKLAELKTHICVTRAFVDNCLQLHEAKRLDSATACMAK YWASELQNSVAYDCVQLHGGWGYMWEYPIAKAYVDARVQP IYGGTNEIMKELIAREIVFDK
- Molecular weight47 kDa including tags
- Amino acids31 to 430
- TagsHis tag N-Terminus
Our Abpromise guarantee covers the use of ab113579 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
- Mass spectrometryMALDI-TOF
- Purity> 85
% by SDS-PAGE.
> 85 % by SDS - PAGE. ab113579 is 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: 0.32% Tris HCl, 10% Glycerol, 0.02% DTT, 0.88% Sodium chloride
- Acyl Coenzyme A dehydrogenase long chain
- Acyl-CoA dehydrogenase long chain
- Long chain acyl CoA dehydrogenase
- Long-chain specific acyl-CoA dehydrogenase
- Long-chain specific acyl-CoA dehydrogenase, mitochondrial
- PathwayLipid metabolism; mitochondrial fatty acid beta-oxidation.
- Involvement in diseaseDefects in ACADL are a cause of acyl-CoA dehydrogenase very long-chain deficiency (ACADVLD) [MIM:201475]. An inborn error of mitochondrial fatty acid beta-oxidation which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form characterized by early onset, high mortality and high incidence of cardiomyopathy; a milder childhood form with later onset, characterized by hypoketotic hypoglycemia, low mortality and rare cardiomyopathy; 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 matrix.
References for Human ACADL full length protein (ab113579)
ab113579 has not yet been referenced specifically in any publications.