Recombinant Human Methylmalonyl Coenzyme A mutase protein (ab114834)

製品の概要

  • 製品名
    Recombinant Human Methylmalonyl Coenzyme A mutase protein
  • タンパク質長
    Full length protein

製品の詳細

  • 由来
    Recombinant
  • 由来
    Wheat germ
  • アミノ酸配列
    • アクセッション番号
    • 生物種
      Human
    • 配列
      MLRAKNQLFLLSPHYLRQVKESSGSRLIQQRLLHQQQPLHPEWAALAKKQ LKGKNPEDLIWHTPEGISIKPLYSKGDTMDLPEELPGVKPFTRGPYPTMY TFRPWTIRQYAGFSTVEESNKFYKDNIKAGQQGLSVAFDLATHRGYDSDN PRVRGDVGMAGVAIDTVEDTKILFDGIPLEKMSVSMTMNGAVIPVLANFI VTGEEQGVPKEKLTGTIQNDILKEFMVRNTYIFPPEPSMKIIADIFEYTA KHMPKFNSISISGYHMQEAGADAILELAYTLADGLEYSRTGLQAGLTIDE FAPRLSFFWGIGMNFYMEIAKMRAGRRLWAHLIEKMFQPKNSKSLLLRAH CQTSGWSLTEQDPYNNIVRTAIEAMAAVFGGTQSLHTNSFDEALGLPTVK SARIARNTQIIIQEESGIPKVADPWGGSYMMECLTNDVYDAALKLINEIE EMGGMAKAVAEGIPKLRIEECAARRQARIDSGSEVIVGVNKYQLEKEDTV EVLAIDNTSVRNRQIEKLKKIKSSRDQALAERCLAALTECAASGDGNILA LAVDASRARCTVGEITDALKKVFGEHKANDRMVSGAYRQEFGESKEITSA IKRVHKFMEREGRRPRLLVAKMGQDGHDRGAKVIATGFADLGFDVDIGPL FQTPREVAQQAVDADVHAVGVNTLAAGHKTLVPELIKELNSLGRPDILVM CGGVIPPQDYEFLFEVGVSNVFGPGTRIPKAAVQVLDDIEKCLEKKQQSV
    • 分子量
      109 kDa including tags
    • 領域
      1 to 750

特性

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.

  • アプリケーション

    ELISA

    SDS-PAGE

    Western blot

  • 製品の状態
    Liquid
  • 備考
    Protein concentration is above or equal to 0.05 mg/ml.
    Best used within three months from the date of receipt.
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

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

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

関連情報

  • 別名
    • MCM
    • Methylmalonyl CoA isomerase
    • Methylmalonyl CoA mutase mitochondrial
    • Methylmalonyl Coenzyme A mutase
    • Methylmalonyl-CoA isomerase
    • Methylmalonyl-CoA mutase
    • mitochondrial
    • Mut
    • MUTA_HUMAN
    see all
  • 機能
    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.
  • 関連疾患
    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.
  • 配列類似性
    Belongs to the methylmalonyl-CoA mutase family.
    Contains 1 B12-binding domain.
  • 細胞内局在
    Mitochondrion matrix.
  • Information by UniProt

画像

  • ab114834 analyzed on a 12.5% SDS-PAGE gel stained with Coomassie Blue.

参考文献

ab114834 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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