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Source Escherichia coli
Amino Acid Sequence
Molecular weight 27 kDa
Amino acids 154 to 404
Tags His-DHFR tag N-Terminus Specifications
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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
Reconstitution Reconstitute with water to desired concentration.
Function Has 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 specificity Detected at comparable levels in all tissues examined (heart, lung, brain, skeletal muscle, pancreas and placenta). Weakly expressed in liver and kidney.
Pathway Amino-acid degradation; L-valine degradation.
Involvement in disease Defects 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 similarities Belongs to the acyl-CoA dehydrogenase family.
Cellular localization Mitochondrion.
Information by UniProt
References for Recombinant Human ACAD8 protein (ab127500)
has not yet been referenced specifically in any publications.
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