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  4. Recombinant Human Probable lysosomal cobalamin transporter (LMBRD1), partial

Recombinant Human Probable lysosomal cobalamin transporter (LMBRD1), partial

  • 中文名称：
    人LMBRD1重组蛋白
  • 货号：
    CSB-YP885730HU
  • 规格：
  • 来源：
    Yeast
  • 其他：
  • 中文名称：
    人LMBRD1重组蛋白
  • 货号：
    CSB-EP885730HU
  • 规格：
  • 来源：
    E.coli
  • 其他：
  • 中文名称：
    人LMBRD1重组蛋白
  • 货号：
    CSB-EP885730HU-B
  • 规格：
  • 来源：
    E.coli
  • 共轭：
    Avi-tag Biotinylated

    E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.

  • 其他：
  • 中文名称：
    人LMBRD1重组蛋白
  • 货号：
    CSB-BP885730HU
  • 规格：
  • 来源：
    Baculovirus
  • 其他：
  • 中文名称：
    人LMBRD1重组蛋白
  • 货号：
    CSB-MP885730HU
  • 规格：
  • 来源：
    Mammalian cell
  • 其他：

产品详情

  • 纯度：
    >85% (SDS-PAGE)
  • 基因名：
    LMBRD1
  • Uniprot No.：
  • 别名：
    HDAg-L-interacting protein NESI; LMBD1_HUMAN; LMBR1 domain-containing protein 1; lmbrd1; Nuclear export signal-interacting protein; Probable lysosomal cobalamin transporter
  • 种属：
    Homo sapiens (Human)
  • 蛋白长度：
    Partial
  • 蛋白标签：
    Tag type will be determined during the manufacturing process.
    The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
  • 产品提供形式：
    Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 复溶：
    We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
  • 储存条件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保质期：
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 货期：
    Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 注意事项：
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet ：
    Please contact us to get it.

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