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  4. MMADHC Antibody, Biotin conjugated

MMADHC Antibody, Biotin conjugated

说明书
  • 中文名称：
    MMADHC兔多克隆抗体, Biotin偶联
  • 货号：
    CSB-PA875665LD01HU
  • 规格：
    ￥880
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) MMADHC Polyclonal antibody
  • Uniprot No.：
    Q9H3L0
  • 基因名：
    MMADHC
  • 别名：
    C2orf25 antibody; cblD antibody; Chromosome 2 open reading frame 25 antibody; CL25022 antibody; Methylmalonic aciduria (cobalamin deficiency) cblD type; with homocystinuria antibody; Methylmalonic aciduria and homocystinuria type D protein antibody; methylmalonic aciduria and homocystinuria type D protein; mitochondrial antibody; mitochondrial antibody; MMAD_HUMAN antibody; Mmadhc antibody; Protein C2orf25; mitochondrial antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Methylmalonic aciduria and homocystinuria type D protein, mitochondrial protein (26-142AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Biotin
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA
  • Protocols：
    ELISA Protocol
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
  • 用途：
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

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MMADHC antibodies

MMADHC Proteins

靶点详情

  • 功能：
    Involved in cobalamin metabolism and trafficking. Plays a role in regulating the biosynthesis and the proportion of two coenzymes, methylcob(III)alamin (MeCbl) and 5'-deoxyadenosylcobalamin (AdoCbl). Promotes oxidation of cob(II)alamin bound to MMACHC. The processing of cobalamin in the cytosol occurs in a multiprotein complex composed of at least MMACHC, MMADHC, MTRR (methionine synthase reductase) and MTR (methionine synthase) which may contribute to shuttle safely and efficiently cobalamin towards MTR in order to produce methionine.
  • 基因功能参考文献：
    1. analysis of crystal structure of the globular C-terminal domain of human CblD, which is sufficient for its interaction with MMADHC or CblC, and for supporting the cytoplasmic cobalamin trafficking pathway PMID: 26364851
    2. specific regions of MMADHC are involved in differential regulation of adenosylcobalamin and methylcobalamin synthesis PMID: 24722857
    3. MMADHC mutations are associated with methylmalonic aciduria and homocystinuria. PMID: 22156578
    4. mutations in a gene designated MMADHC (currently named C2orf25) are responsible for the cblD defect in vitamin B12 metabolism; various mutations are associated with each of the three biochemical phenotypes of the disorder PMID: 18385497
  • 相关疾病：
    Methylmalonic aciduria and homocystinuria type cblD (MMAHCD)
  • 亚细胞定位：
    Cytoplasm. Mitochondrion.
  • 组织特异性：
    Widely expressed at high levels.
  • 数据库链接：

    HGNC: 25221

    OMIM: 277410

    KEGG: hsa:27249

    STRING: 9606.ENSP00000301920

    UniGene: Hs.5324

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