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Storage instructions Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Storage buffer Preservative: 0.01% Thimerosal (merthiolate)
Constituents: 10% Glycerol, 0.1M Tris, 0.1M Glycine, pH 7.0
Concentration information loading...
Purity Immunogen affinity purified
Abpromise guarantee covers the use of
in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
1/500 - 1/3000. Predicted molecular weight: 45 kDa.
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
ACAD 8 antibody
Anti-ACAD8 antibody images
References for Anti-ACAD8 antibody (ab96567)
has not yet been referenced specifically in any publications.
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