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Overview

  • Product nameACAD8 protein (His-DHFR tag)
  • Protein descriptionRecombinant fragment corresponding to amino acids 154-404 of Human ACAD8 with N terminal His-DHFR tag; Predicted MWt 26.9 kDA excluding tag (BC001964).
  • Uniprot accessionQ9UKU7
  • Molecular weight26.900kDa
  • Protein length251 amino acids
  • Expression hostE. coli

    • Properties

  • Purification notesPurity determined to be 90% by SDS-PAGE.
  • E.C. Number1.3.99.-
  • FormLyophilised:Reconstitute with water to desired concentration.
  • Storage instructionsShipped at 4°C. Store at -20ºC.
  • Storage bufferConstituents: 0.32% Tris HCl, 0.58% Sodium chloride
  • Concentration information loading...
  • Research Areas

    • Applications

    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.

    Application Notes
    SDS-PAGE SDS-PAGE: Use at an assay dependent dilution.

    Protein info

    • Alternative names
        ACAD 8ACAD-8ACAD8
        ACAD8_HUMANActivator-recruited cofactor 42 kDa componentAcyl CoA dehydrogenase family member 8Acyl Coenzyme A dehydrogenase family member 8Acyl-CoA dehydrogenase family member 8ARC42FLJ22590IBDIsobutyryl-CoA dehydrogenaseIsobutyryl-CoA dehydrogenase, mitochondrialmitochondrial
      see all
  • 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.

    • Target information above from: UniProt accession Q9UKU7 The UniProt Consortium
    The Universal Protein Resource (UniProt) in 2010
    Nucleic Acids Res. 38:D142-D148 (2010) .

    Information by UniProt

    References for ACAD8 protein (His-DHFR tag) (ab127500)

    ab127500 has not yet been referenced specifically in any publications.

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