Recombinant Human ACADSB protein (ab127117)

Overview

Description

  • Nature
    Recombinant
  • Source
    Escherichia coli
  • Amino Acid Sequence
    • Accession
    • Species
      Human
    • Molecular weight
      24 kDa
    • Amino acids
      180 to 402
    • Tags
      His-DHFR tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab127117 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
    • 2 MEBCAD
    • 2 methyl branched chain acyl CoA dehydrogenase
    • 2 methylbutyryl CoA dehydrogenase
    • 2 methylbutyryl coenzyme A dehydrogenase
    • 2-MEBCAD
    • 2-methyl branched chain acyl-CoA dehydrogenase
    • 2-methylbutyryl-CoA dehydrogenase
    • 2-methylbutyryl-coenzyme A dehydrogenase
    • ACAD7
    • ACADSB
    • ACDSB_HUMAN
    • acyl CoA dehydrogenase, short/branched chain
    • acyl Coenzyme A dehydrogenase short branched chain
    • mitochondrial
    • OTTHUMP00000020685
    • OTTHUMP00000046795
    • SBCAD
    • Short/branched chain specific acyl-CoA dehydrogenase
    • short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
    see all
  • Function
    Has greatest activity toward short branched chain acyl-CoA derivative such as (s)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2-methylhexanoyl-CoA as well as toward short straight chain acyl-CoAs such as butyryl-CoA and hexanoyl-CoA. Can use valproyl-CoA as substrate and may play a role in controlling the metabolic flux of valproic acid in the development of toxicity of this agent.
  • Tissue specificity
    Ubiquitous.
  • Pathway
    Lipid metabolism; mitochondrial fatty acid beta-oxidation.
  • Involvement in disease
    Defects in ACADSB are the cause of short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD) [MIM:610006]; also known as 2-methylbutyryl-CoA dehydrogenase deficiency or 2-methylbutyryl glycinuria. SBCADD is an autosomal recessive disorder and consists of a defect in catabolism of L-isoleucine which is characterized by an increase of 2-methylbutyrylglycine and 2-methylbutyrylcarnitine in blood and urine. Affected individuals have seizures and psychomotor delay as the main clinical features.
  • Sequence similarities
    Belongs to the acyl-CoA dehydrogenase family.
  • Cellular localization
    Mitochondrion matrix.
  • Information by UniProt

References for Recombinant Human ACADSB protein (ab127117)

ab127117 has not yet been referenced specifically in any publications.

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