Recombinant人Methylmalonyl Coenzyme A mutase protein (ab114834)

概述

  • 产品名称Recombinant人Methylmalonyl Coenzyme A mutase protein
  • 蛋白长度Full length protein

描述

  • 性质Recombinant
  • 来源Wheat germ
  • 氨基酸序列
    • AccessionP22033
    • 种属Human
    • 序列MLRAKNQLFLLSPHYLRQVKESSGSRLIQQRLLHQQQPLHPEWAALAKKQ LKGKNPEDLIWHTPEGISIKPLYSKGDTMDLPEELPGVKPFTRGPYPTMY TFRPWTIRQYAGFSTVEESNKFYKDNIKAGQQGLSVAFDLATHRGYDSDN PRVRGDVGMAGVAIDTVEDTKILFDGIPLEKMSVSMTMNGAVIPVLANFI VTGEEQGVPKEKLTGTIQNDILKEFMVRNTYIFPPEPSMKIIADIFEYTA KHMPKFNSISISGYHMQEAGADAILELAYTLADGLEYSRTGLQAGLTIDE FAPRLSFFWGIGMNFYMEIAKMRAGRRLWAHLIEKMFQPKNSKSLLLRAH CQTSGWSLTEQDPYNNIVRTAIEAMAAVFGGTQSLHTNSFDEALGLPTVK SARIARNTQIIIQEESGIPKVADPWGGSYMMECLTNDVYDAALKLINEIE EMGGMAKAVAEGIPKLRIEECAARRQARIDSGSEVIVGVNKYQLEKEDTV EVLAIDNTSVRNRQIEKLKKIKSSRDQALAERCLAALTECAASGDGNILA LAVDASRARCTVGEITDALKKVFGEHKANDRMVSGAYRQEFGESKEITSA IKRVHKFMEREGRRPRLLVAKMGQDGHDRGAKVIATGFADLGFDVDIGPL FQTPREVAQQAVDADVHAVGVNTLAAGHKTLVPELIKELNSLGRPDILVM CGGVIPPQDYEFLFEVGVSNVFGPGTRIPKAAVQVLDDIEKCLEKKQQSV
    • 分子量109 kDa including tags
    • 氨基酸1 to 750

技术指标

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.

  • 应用

    ELISA

    SDS-PAGE

    Western blot

  • 形式Liquid
  • 补充说明Protein concentration is above or equal to 0.05 mg/ml.
    Best used within three months from the date of receipt.
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    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

常规信息

  • 别名
    • MCM
    • Methylmalonyl CoA isomerase
    • Methylmalonyl CoA mutase mitochondrial
    • Methylmalonyl Coenzyme A mutase
    • Methylmalonyl-CoA isomerase
    • Methylmalonyl-CoA mutase
    • mitochondrial
    • Mut
    • MUTA_HUMAN
    see all
  • 功能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.
  • 疾病相关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.
  • 序列相似性Belongs to the methylmalonyl-CoA mutase family.
    Contains 1 B12-binding domain.
  • 细胞定位Mitochondrion matrix.
  • Information by UniProt

Recombinant Human Methylmalonyl Coenzyme A mutase protein 图像

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

Recombinant Human Methylmalonyl Coenzyme A mutase protein (ab114834)参考文献

ab114834 has not yet been referenced specifically in any publications.

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