Human ACADSB protein fragment (ab127117)
- Product nameHuman ACADSB protein fragmentSee all ACADSB proteins and peptides ...
- DescriptionRecombinant, His-DHFR tag
- SourceE. coli
- Amino Acid Sequence
- Molecular weight24 kDa
- Amino acids180 to 402
- TagsHis-DHFR tag N-Terminus
Our Abpromise guarantee covers the use of ab127117 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
- Concentration information loading...
Preparation and Storage
- Stability and Storage
Shipped at 4°C. Store at -20ºC.
Constituents: 0.32% Tris HCl, 0.58% Sodium chloride
- ReconstitutionReconstitute with water to desired concentration
- 2 MEBCAD
- 2 methyl branched chain acyl CoA dehydrogenase
- 2 methylbutyryl CoA dehydrogenase
- 2 methylbutyryl coenzyme A dehydrogenase
- 2-methyl branched chain acyl-CoA dehydrogenase
- 2-methylbutyryl-CoA dehydrogenase
- 2-methylbutyryl-coenzyme A dehydrogenase
- acyl CoA dehydrogenase, short/branched chain
- acyl Coenzyme A dehydrogenase short branched chain
- Short/branched chain specific acyl-CoA dehydrogenase
- short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
- FunctionHas greatest activity toward short branched chain acyl-CoA derivative such as (s)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2-methylhexanoyl-CoA as well as toward short straight chain acyl-CoAs such as butyryl-CoA and hexanoyl-CoA. Can use valproyl-CoA as substrate and may play a role in controlling the metabolic flux of valproic acid in the development of toxicity of this agent.
- Tissue specificityUbiquitous.
- PathwayLipid metabolism; mitochondrial fatty acid beta-oxidation.
- Involvement in diseaseDefects in ACADSB are the cause of short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD) [MIM:610006]; also known as 2-methylbutyryl-CoA dehydrogenase deficiency or 2-methylbutyryl glycinuria. SBCADD is an autosomal recessive disorder and consists of a defect in catabolism of L-isoleucine which is characterized by an increase of 2-methylbutyrylglycine and 2-methylbutyrylcarnitine in blood and urine. Affected individuals have seizures and psychomotor delay as the main clinical features.
- Sequence similaritiesBelongs to the acyl-CoA dehydrogenase family.
- Cellular localizationMitochondrion matrix.
References for Human ACADSB protein fragment (ab127117)
ab127117 has not yet been referenced specifically in any publications.