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    recombinant-human-methylmalonyl-coenzyme-a-mutase-protein-ab114834.pdf

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Recombinant Human Methylmalonyl Coenzyme A mutase protein (ab114834)

  • Datasheet
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SDS-PAGE - Recombinant Human Methylmalonyl Coenzyme A mutase protein (ab114834)

    Key features and details

    • Expression system: Wheat germ
    • Suitable for: ELISA, SDS-PAGE, WB

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    Description

    • Product name

      Recombinant Human Methylmalonyl Coenzyme A mutase protein
      See all Methylmalonyl Coenzyme A mutase proteins and peptides
    • Expression system

      Wheat germ
    • Accession

      P22033
    • Protein length

      Full length protein
    • Animal free

      No
    • Nature

      Recombinant
      • Species

        Human
      • Sequence

        MLRAKNQLFLLSPHYLRQVKESSGSRLIQQRLLHQQQPLHPEWAALAKKQ LKGKNPEDLIWHTPEGISIKPLYSKGDTMDLPEELPGVKPFTRGPYPTMY TFRPWTIRQYAGFSTVEESNKFYKDNIKAGQQGLSVAFDLATHRGYDSDN PRVRGDVGMAGVAIDTVEDTKILFDGIPLEKMSVSMTMNGAVIPVLANFI VTGEEQGVPKEKLTGTIQNDILKEFMVRNTYIFPPEPSMKIIADIFEYTA KHMPKFNSISISGYHMQEAGADAILELAYTLADGLEYSRTGLQAGLTIDE FAPRLSFFWGIGMNFYMEIAKMRAGRRLWAHLIEKMFQPKNSKSLLLRAH CQTSGWSLTEQDPYNNIVRTAIEAMAAVFGGTQSLHTNSFDEALGLPTVK SARIARNTQIIIQEESGIPKVADPWGGSYMMECLTNDVYDAALKLINEIE EMGGMAKAVAEGIPKLRIEECAARRQARIDSGSEVIVGVNKYQLEKEDTV EVLAIDNTSVRNRQIEKLKKIKSSRDQALAERCLAALTECAASGDGNILA LAVDASRARCTVGEITDALKKVFGEHKANDRMVSGAYRQEFGESKEITSA IKRVHKFMEREGRRPRLLVAKMGQDGHDRGAKVIATGFADLGFDVDIGPL FQTPREVAQQAVDADVHAVGVNTLAAGHKTLVPELIKELNSLGRPDILVM CGGVIPPQDYEFLFEVGVSNVFGPGTRIPKAAVQVLDDIEKCLEKKQQSV
      • Predicted molecular weight

        109 kDa including tags
      • Amino acids

        1 to 750

    Associated products

    • Related Products

      • Anti-Methylmalonyl Coenzyme A mutase antibody (ab67869)

    Specifications

    Our Abpromise guarantee covers the use of ab114834 in the following tested applications.

    The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

    • Applications

      ELISA

      SDS-PAGE

      Western blot

    • Form

      Liquid
    • Concentration information loading...

    Preparation and Storage

    • Stability and Storage

      Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

      pH: 8.00
      Constituents: 0.3% Glutathione, 0.79% Tris HCl

    General Info

    • Alternative names

      • MCM
      • Methylmalonyl CoA isomerase
      • Methylmalonyl CoA mutase mitochondrial
      • Methylmalonyl Coenzyme A mutase
      • Methylmalonyl-CoA isomerase
      • Methylmalonyl-CoA mutase
      • mitochondrial
      • Mut
      • MUTA_HUMAN
      see all
    • Function

      Involved in the degradation of several amino acids, odd-chain fatty acids and cholesterol via propionyl-CoA to the tricarboxylic acid cycle. MCM has different functions in other species.
    • Involvement in disease

      Defects in MUT are the cause of methylmalonic aciduria type mut (MMAM) [MIM:251000]. MMAM is an often fatal disorder of organic acid metabolism. Common clinical features include lethargy, vomiting, failure to thrive, hypotonia, neurological deficit and early death. Two forms of the disease are distinguished by the presence (mut-) or absence (mut0) of residual enzyme activity. Mut0 patients have more severe neurological manifestations of the disease than do MUT- patients. MMAM is unresponsive to vitamin B12 therapy.
    • Sequence similarities

      Belongs to the methylmalonyl-CoA mutase family.
      Contains 1 B12-binding domain.
    • Cellular localization

      Mitochondrion matrix.
    • Target information above from: UniProt accession P22033 The UniProt Consortium
      The Universal Protein Resource (UniProt) in 2010
      Nucleic Acids Res. 38:D142-D148 (2010) .

      Information by UniProt

    Images

    • SDS-PAGE - Recombinant Human Methylmalonyl Coenzyme A mutase protein (ab114834)
      SDS-PAGE - Recombinant Human Methylmalonyl Coenzyme A mutase protein (ab114834)
      ab114834 analyzed on a 12.5% SDS-PAGE gel stained with Coomassie Blue.

    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

    Datasheets and documents

    • Datasheet download

      Download

    References (0)

    Publishing research using ab114834? Please let us know so that we can cite the reference in this datasheet.

    ab114834 has not yet been referenced specifically in any publications.

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