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
    • Accession
    • 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

图片

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

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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