Overview

Description

  • Nature
    Recombinant
  • Source
    Escherichia coli
  • Amino Acid Sequence
    • Accession
    • Species
      Human
    • Molecular weight
      27 kDa
    • Amino acids
      154 to 404
    • Tags
      His-DHFR tag N-Terminus

Specifications

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.

  • Applications

    SDS-PAGE

  • Form
    Lyophilised
  • 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.

General Info

  • Alternative names
    • ACAD 8
    • ACAD-8
    • ACAD8
    • ACAD8_HUMAN
    • 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
    • ARC42
    • FLJ22590
    • IBD
    • Isobutyryl-CoA dehydrogenase
    • Isobutyryl-CoA dehydrogenase, mitochondrial
    • mitochondrial
    see all
  • 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)

ab127500 has not yet been referenced specifically in any publications.

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