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LMBRD1 Antibody

  • 货号：
    CSB-PA885730LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Western Blot
      Positive WB detected in: Mouse liver tissue, Mouse kidney tissue
      All lanes: LMBRD1 antibody at 4μg/ml
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 62, 45, 54, 22 kDa
      Observed band size: 62, 54 kDa
    • Immunohistochemistry of paraffin-embedded human brain tissue using CSB-PA885730LA01HU at dilution of 1:100
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) LMBRD1 Polyclonal antibody
  • Uniprot No.：
    Q9NUN5
  • 基因名：
    LMBRD1
  • 别名：
    HDAg-L-interacting protein NESI antibody; LMBD1_HUMAN antibody; LMBR1 domain-containing protein 1 antibody; lmbrd1 antibody; Nuclear export signal-interacting protein antibody; Probable lysosomal cobalamin transporter antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human, Mouse
  • 免疫原：
    Recombinant Human Probable lysosomal cobalamin transporter protein (6-100AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，LMBRD1 Antibody (CSB-PA885730LA01HU)，的标记方式是Non-conjugated。对于LMBRD1 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-PA885730LB01HU LMBRD1 Antibody, HRP conjugated ELISA
    FITC CSB-PA885730LC01HU LMBRD1 Antibody, FITC conjugated
    Biotin CSB-PA885730LD01HU LMBRD1 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, WB, IHC
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:5000
    IHC 1:20-1:200
  • 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：
    Lysosomal membrane chaperone required to export cobalamin (vitamin B12) from the lysosome to the cytosol, allowing its conversion to cofactors. Targets ABCD4 transporter from the endoplasmic reticulum to the lysosome. Then forms a complex with lysosomal ABCD4 and cytoplasmic MMACHC to transport cobalamin across the lysosomal membrane. Acts as an adapter protein which plays an important role in mediating and regulating the internalization of the insulin receptor (INSR). Involved in clathrin-mediated endocytosis of INSR via its interaction with adapter protein complex 2. Essential for the initiation of gastrulation and early formation of mesoderm structures during embryogenesis.; (Microbial infection) May play a role in the assembly of hepatitis delta virus (HDV).
  • 基因功能参考文献：
    1. endogenous ABCD4 was localized to both lysosomes and the ER, and its lysosomal localization was disturbed by knockout of LMBRD1 PMID: 27456980
    2. Data suggest that ABCD4 lysosomal targeting depends on co-expression of and interaction with LMBRD1; mutations in LMBRD1 and ABCD4 that result in cobalamin metabolism disorders cblF and cblJ (or mutations in ATPase domain) disrupt interactions between LMBRD1 and ABCD4. (LMBRD1 = nuclear export signal-interacting protein; ABCD4 = ATP-binding cassette, sub-family D (ALD), member 4) PMID: 28572511
    3. Results propose a model whereby membrane-bound LMBD1 and ABCD4 facilitate the vectorial delivery of lysosomal vitamin B12 to cytoplasmic MMACHC. PMID: 25535791
    4. LMBD1 plays an imperative role in mediating and regulating the endocytosis of the IR. PMID: 24078630
    5. These data indicate that by forming complexes with lamin A/C and nucleoporins, NESI facilitates the CRM1-independent nuclear export of large hepatitis delta antigen. PMID: 23175358
    6. novel mutations in LMBRD1 in three patients PMID: 21303734
    7. LMBRD1: the gene for the cblF defect of vitamin B metabolism. PMID: 20446115
    8. a LMBRD1 mutation causes the cblF defect of vitamin B(12) metabolism in a Turkish patient [case report] PMID: 20127417
    9. LMBRD1 is the gene underlying the cblF defect of cobalamin metabolism and suggests that LMBD1 is a lysosomal membrane exporter for cobalamin. PMID: 19136951

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  • 相关疾病：
    Methylmalonic aciduria and homocystinuria type cblF (MMAHCF)
  • 亚细胞定位：
    Endoplasmic reticulum membrane. Lysosome membrane; Multi-pass membrane protein. Cell membrane; Multi-pass membrane protein. Cytoplasmic vesicle, clathrin-coated vesicle.
  • 蛋白家族：
    LIMR family, LMBRD1 subfamily
  • 组织特异性：
    Isoform 3 is expressed in liver.
  • 数据库链接：

    HGNC: 23038

    OMIM: 277380

    KEGG: hsa:55788

    STRING: 9606.ENSP00000359609

    UniGene: Hs.271643