Recombinant Human ACADSB protein (ab130042)

Overview

Description

  • Nature
    Recombinant
  • Source
    Escherichia coli
  • Amino Acid Sequence
    • Accession
    • Species
      Human
    • Sequence
      MGSSHHHHHH SSGLVPRGSH MGSHMKSSQS EALLNITNNG IHFAPLQTFT DEEMMIKSSV KKFAQEQIAP LVSTMDENSK MEKSVIQGLF QQGLMGIEVD PEYGGTGASF LSTVLVIEEL AKVDASVAVF CEIQNTLINT LIRKHGTEEQ KATYLPQLTT EKVGSFCLSE AGAGSDSFAL KTRADKEGDY YVLNGSKMWI SSAEHAGLFL VMANVDPTIG YKGITSFLVD RDTPGLHIGK PENKLGLRAS STCPLTFENV KVPEANILGQ IGHGYKYAIG SLNEGRIGIA AQMLGLAQGC FDYTIPYIKE RIQFGKRLFD FQGLQHQVAH VATQLEAARL LTYNAARLLE AGKPFIKEAS MAKYYASEIA GQTTSKCIEW MGGVGYTKDY PVEKYFRDAK IGTIYEGASN IQLNTIAKHI DAEY
    • Molecular weight
      46 kDa including tags
    • Amino acids
      34 to 432
    • Tags
      His tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab130042 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

  • Purity
    >90% by SDS-PAGE.
    ab130042 is purified using conventional chromatography techniques.
  • Form
    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.

    pH: 8.00
    Constituents: 0.02% DTT, 0.32% Tris HCl, 10% Glycerol, 0.58% Sodium chloride

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

Recombinant Human ACADSB protein images

  • 15% SDS-PAGE showing ab130042 (3 µg).

References for Recombinant Human ACADSB protein (ab130042)

ab130042 has not yet been referenced specifically in any publications.

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