ACAD8 protein (His-DHFR tag) (ab127500)
- Product nameACAD8 protein (His-DHFR tag)See all ACAD8 proteins and peptides ...
- SourceE. coli
- Amino Acid Sequence
- Molecular weight27 kDa
- Amino acids154 to 404
- TagsHis-DHFR tag N-Terminus
Our Abpromise guarantee covers the use of ab127500 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.
- ACAD 8
- Activator-recruited cofactor 42 kDa component
- Acyl CoA dehydrogenase family member 8
- Acyl Coenzyme A dehydrogenase family member 8
- Acyl-CoA dehydrogenase family member 8
- Isobutyryl-CoA dehydrogenase
- Isobutyryl-CoA dehydrogenase, mitochondrial
- FunctionHas very high activity toward isobutyryl-CoA. Is an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Plays a role in transcriptional coactivation within the ARC complex.
- Tissue specificityDetected at comparable levels in all tissues examined (heart, lung, brain, skeletal muscle, pancreas and placenta). Weakly expressed in liver and kidney.
- PathwayAmino-acid degradation; L-valine degradation.
- Involvement in diseaseDefects in ACAD8 are the cause of isobutyryl-CoA dehydrogenase deficiency (IBDD) [MIM:611283]. The symptoms of IBDD generally appear until late in infancy or in childhood and can include poor feeding and growth (failure to thrive), a weakened and enlarged heart (dilated cardiomyopathy), seizures, and low numbers of red blood cells (anemia).
- Sequence similaritiesBelongs to the acyl-CoA dehydrogenase family.
- Cellular localizationMitochondrion.
References for ACAD8 protein (His-DHFR tag) (ab127500)
ab127500 has not yet been referenced specifically in any publications.