日韩在线视频不卡-日一区二区三区-天堂国产+人+综合+亚洲欧美-精品国产一区二区三区久久狼-国产成人无码18禁午夜福利网址-国产在线拍偷自揄拍精品-九色91丨porny丨丝袜-久久天天躁狠狠躁夜夜av-99蜜桃臀久久久欧美精品-成人一区二区视频-狠狠操人人干-40岁干柴烈火少妇高潮不断-国产福利不卡-伊人98-丰满多毛的陰户视频-日韩一二三四五区-国产专区一线二线三线码-好男人社区影院www-国产精品2019-欧美三级一区

Your Good Partner in Biology Research

  1. Home
  2. 抗體
  3. 多克隆抗體
  4. ABCD4 Antibody

ABCD4 Antibody

  • 中文名稱：
    ABCD4兔多克隆抗體
  • 貨號：
    CSB-PA970746
  • 規格：
    ￥2024
  • 圖片：
    • Western blot analysis of extracts from HepG2 cells, using ABCD4 antibody.
  • 其他：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) ABCD4 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    ABCD4
  • 別名：
    ABCD4; PXMP1L; Lysosomal cobalamin transporter ABCD4; ATP-binding cassette sub-family D member 4; PMP70-related protein; P70R; Peroxisomal membrane protein 1-like; PXMP1-L; Peroxisomal membrane protein 69; PMP69
  • 宿主：
    Rabbit
  • 反應種屬：
    Human
  • 免疫原：
    Synthesized peptide derived from internal of Human ABCD4.
  • 免疫原種屬：
    Homo sapiens (Human)
  • 克隆類型：
    Polyclonal
  • 純化方式：
    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 產品提供形式：
    Liquid
  • 應用范圍：
    ELISA,WB
  • 推薦稀釋比：
    Application Recommended Dilution
    WB 1:500-1:3000
  • Protocols：
  • 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 protein that transports cobalamin (Vitamin B12) from the lysosomal lumen to the cytosol in an ATP-dependent manner. Targeted by LMBRD1 lysosomal chaperone from the endoplasmic reticulum to the lysosomal membrane. Then forms a complex with lysosomal chaperone LMBRD1 and cytosolic MMACHC to transport cobalamin across the lysosomal membrane.
  • 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. mutations altering the putative ATPase domain of ABCD4 affect its function, suggesting that the ATPase activity of ABCD4 may be involved in intracellular processing of vitamin B12. PMID: 22922874
    5. Defects in ABCB4 have been found to cause progressive familial intrahepatic cholestasis type 3. PMID: 21514256
    6. expression tends to be correlated with the severity of X-linked adrenoleukodystrophy PMID: 15800013

    顯示更多

    收起更多

  • 相關疾病：
    Methylmalonic aciduria and homocystinuria type cblJ (MAHCJ)
  • 亞細胞定位：
    Endoplasmic reticulum membrane; Multi-pass membrane protein. Lysosome membrane; Multi-pass membrane protein.
  • 蛋白家族：
    ABC transporter superfamily, ABCD family, Peroxisomal fatty acyl CoA transporter (TC 3.A.1.203) subfamily
  • 組織特異性：
    Ubiquitous.
  • 數據庫鏈接：

    HGNC: 68

    OMIM: 603214

    KEGG: hsa:5826

    STRING: 9606.ENSP00000349396

    UniGene: Hs.94395