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NPC1 抗体 (AA 34-174)

NPC1 适用: 人 ELISA, IHC, FACS 宿主: 小鼠 Monoclonal 8D10G3 unconjugated
产品编号 ABIN1724840
发货至: 中国
  • 抗原 See all NPC1 抗体
    NPC1 (Niemann-Pick Disease, Type C1 (NPC1))
    抗原表位
    • 6
    • 5
    • 5
    • 4
    • 4
    • 3
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    AA 34-174
    适用
    • 43
    • 17
    • 13
    • 1
    宿主
    • 39
    • 4
    • 2
    小鼠
    克隆类型
    • 34
    • 11
    单克隆
    标记
    • 20
    • 4
    • 3
    • 2
    • 2
    • 2
    • 2
    • 2
    • 2
    • 2
    • 2
    • 2
    This NPC1 antibody is un-conjugated
    应用范围
    • 27
    • 26
    • 18
    • 11
    • 6
    • 5
    • 4
    • 1
    • 1
    • 1
    • 1
    ELISA, Immunohistochemistry (IHC), Flow Cytometry (FACS)
    原理
    NPC1 Antibody
    纯化方法
    Purified antibody
    免疫原
    Purified recombinant fragment of human NPC1 (AA: 34-174) expressed in E. Coli.
    克隆位点
    8D10G3
    亚型
    IgG1
  • 应用备注

    ELISA: 1/10000

    FCM: 1/200 - 1/400

    限制
    仅限研究用
  • 状态
    Liquid
    缓冲液
    Purified antibody in PBS with 0.05 % sodium azide.
    储存液
    Sodium azide
    注意事项
    This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
    储存条件
    4 °C,-20 °C
    储存方法
    Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
  • Morales, Amigo, Balboa, Acuña, Castro, Molina, Miquel, Nervi, Rigotti, Zanlungo: "Deficiency of Niemann-Pick C1 protein protects against diet-induced gallstone formation in mice." in: Liver international : official journal of the International Association for the Study of the Liver, Vol. 30, Issue 6, pp. 887-97, (2010) (PubMed).

    Xu, Zhou, Xia, Xia, Chen, Duan, Luo: "Defects of synaptic vesicle turnover at excitatory and inhibitory synapses in Niemann-Pick C1-deficient neurons." in: Neuroscience, Vol. 167, Issue 3, pp. 608-20, (2010) (PubMed).

  • 抗原
    NPC1 (Niemann-Pick Disease, Type C1 (NPC1))
    别名
    NPC1 (NPC1 产品)
    背景

    Description: This gene encodes a large protein that resides in the limiting membrane of endosomes and lysosomes and mediates intracellular cholesterol trafficking via binding of cholesterol to its N-terminal domain. It is predicted to have a cytoplasmic C-terminus, 13 transmembrane domains, and 3 large loops in the lumen of the endosome - the last loop being at the N-terminus. This protein transports low-density lipoproteins to late endosomal/lysosomal compartments where they are hydrolized and released as free cholesterol. Defects in this gene cause Niemann-Pick type C disease, a rare autosomal recessive neurodegenerative disorder characterized by over accumulation of cholesterol and glycosphingolipids in late endosomal/lysosomal compartments.

    Aliases: NPC

    分子量
    142.2kDa
    基因ID
    4864
    HGNC
    4864
    UniProt
    O15118
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