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MMADHC Antibody

  • 货号：
    CSB-PA875665LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • IHC image of CSB-PA875665LA01HU diluted at 1:300 and staining in paraffin-embedded human glioma performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
    • IHC image of CSB-PA875665LA01HU diluted at 1:300 and staining in paraffin-embedded human prostate cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
    • Immunofluorescence staining of HepG2 cells with CSB-PA875665LA01HU at 1:100, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
  • 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：
    Non-conjugated

    本页面中的产品，MMADHC Antibody (CSB-PA875665LA01HU)，的标记方式是Non-conjugated。对于MMADHC Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA875665LB01HU MMADHC Antibody, HRP conjugated ELISA
    FITC CSB-PA875665LC01HU MMADHC Antibody, FITC conjugated
    Biotin CSB-PA875665LD01HU MMADHC Antibody, Biotin conjugated ELISA
  • 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, IHC, IF
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:200-1:500
    IF 1:50-1:200
  • Protocols：
  • 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.

产品评价

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