全部产品分类
品牌 |
Leading Biology | 货号 |
APR07141G |
产品分类 |
Monoclonal Antibodies | 研究领域 |
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产品概述 |
We constantly strive to ensure we provide our customers with the best antibodies. As a result of this work we offer this antibody in purified format.
We are in the process of updating our datasheets. If you have any questions regarding this update, please feel free to contact our technical support team.
This product is a high quality NPC1 antibody (monoclonal) (M02).
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分子量 |
142167 Da
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宿主 |
mouse
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种属反应性 |
Human
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靶点 |
NPC1 (AAH63302, 151 a.a. ~ 250 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
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克隆号 |
4H2
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亚型 |
IgG2a Kappa
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基因ID |
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UniProt ID |
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总结 |
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.
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形式 |
Clear, colorless solution in phosphate buffered saline, pH 7.2 .
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储存条件 |
Store at +4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.
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应用 |
WB, E
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稀释方法 |
WB~~1:500~1000
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别名 |
Niemann-Pick C1 protein, NPC1
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图像 |
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说明书 |
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数量 |
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