Recombinant Human Methylmalonyl Coenzyme A mutase protein (ab114834)

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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

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.
  • 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

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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