在线日韩日本国产亚洲丨少妇伦子伦情品无吗丨欧美性猛交xxxx免费看蜜桃丨精品人妻系列无码一区二区三区丨亚洲精品无码不卡在线播放

Your Good Partner in Biology Research

  1. Home
  2. 抗體
  3. 多克隆抗體
  4. LMBRD1 Antibody, FITC conjugated

LMBRD1 Antibody, FITC conjugated

  • 中文名稱：
    LMBRD1兔多克隆抗體, FITC偶聯
  • 貨號：
    CSB-PA885730LC01HU
  • 規格：
    ￥880
  • 其他：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) LMBRD1 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    LMBRD1
  • 別名：
    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
  • 反應種屬：
    Human
  • 免疫原：
    Recombinant Human Probable lysosomal cobalamin transporter protein (6-100AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    FITC
  • 克隆類型：
    Polyclonal
  • 抗體亞型：
    IgG
  • 純化方式：
    >95%, Protein G purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
  • 產品提供形式：
    Liquid
  • 儲存條件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期：
    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.

產品評價

靶點詳情

  • 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).
  • 基因功能參考文獻：
    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

    顯示更多

    收起更多

  • 相關疾病：
    Methylmalonic aciduria and homocystinuria type cblF (MMAHCF)
  • 亞細胞定位：
    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
  • 組織特異性：
    Isoform 3 is expressed in liver.
  • 數據庫鏈接：

    HGNC: 23038

    OMIM: 277380

    KEGG: hsa:55788

    STRING: 9606.ENSP00000359609

    UniGene: Hs.271643



主站蜘蛛池模板： 天天躁夜夜躁狠狠躁2021| 亚洲国产精品成人一区二区在线 | 国产香港明星裸体xxxx视频| 久久视频这里只有精品在线观看| 亚洲区激情区无码区日韩区| 日韩免费视频| 色五月五月丁香亚洲综合网| 国产天美传媒性色av出轨| 久久综合av色老头免费观看| 越南女子杂交内射bbwxz| 色噜噜av男人的天堂| 久久久婷婷五月亚洲97号色| 熟女毛多熟妇人妻在线视频| 99国产欧美久久久精品| 亚洲精品欧美二区三区中文字幕| 精品久久久久久久久久中文字幕| 亚洲国产成人av在线观看| 日韩精品无码人妻一区二区三区| 国产精品无码a∨麻豆| 久久精品无码精品免费专区| 美女张开腿黄网站免费下载| 9l国产精品久久久久麻豆| 久久麻豆成人精品av| 激情综合色综合啪啪五月丁香 | 久久99九九精品久久久久蜜桃| 窝窝午夜理论片影院| 国产成人精品男人的天堂| 久久久综合九色综合鬼色| 18禁成年无码免费网站| 少妇高潮一区二区三区99| 无码av天天av天天爽| 国语对白做受xxxxx在| 国精一二二产品无人区免费应用| 国产亚洲精品a在线无码| 亚洲va天堂va欧美片a在线| 久久国产亚洲精选av| 日本乱偷人妻中文字幕在线| 国产伦子系列沙发午睡| 性无码专区一色吊丝中文字幕| 浪潮av激情高潮国产精品| 麻花豆传媒剧国产免费mv入口|