Recombinant Human SBCAD protein (ab130042)

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SDS-PAGE - Recombinant Human SBCAD protein (ab130042)

    Key features and details

    • Expression system: Escherichia coli
    • Purity: > 90% SDS-PAGE
    • Tags: His tag N-Terminus
    • Suitable for: SDS-PAGE

    Description

    • Product name

      Recombinant Human SBCAD protein

    • Purity

      > 90 % SDS-PAGE.
      ab130042 is purified using conventional chromatography techniques.

    • Expression system

      Escherichia coli

    • Accession

      P45954

    • Protein length

      Full length protein

    • Animal free

      No

    • Nature

      Recombinant

      • 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

      • Predicted 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

    • Form

      Liquid

    • Additional notes

      Previously labelled as ACADSB.

    • 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: 0.58% Sodium chloride, 10% Glycerol (glycerin, glycerine), 0.32% Tris HCl, 0.02% DTT

    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

    Images

    • SDS-PAGE - Recombinant Human SBCAD protein (ab130042)
      SDS-PAGE - Recombinant Human SBCAD protein (ab130042)
      15% SDS-PAGE showing ab130042 (3 µg).

    Protocols

    To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.

    Click here to view the general protocols

    References (0)

    Publishing research using ab130042? Please let us know so that we can cite the reference in this datasheet.

    ab130042 has not yet been referenced specifically in any publications.

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