产品详情
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Product Name
Niemann Pick C1 Mouse mAb
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Gene ID
4864
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SwissProt ID
O15118
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Gene Name
NPC1
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Alternative Names
NPC
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Background
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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Research Field
Neuroscience
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Product Categories
Primary antibody
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Host
Mouse
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Reactivity
Human
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Application
IHC-P,FC,ELISA
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Dilution Ratio
IHC: 1/100-1/200 FC: 1/50-1/100 ELISA: 1/10000
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Molecular Weight
-
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Clonality
Monoclonal Antibody
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Clonality No.
1P4-L8-C8
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Isotype
Mouse IgG1
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Immunogen
Purified recombinant fragment of human NPC1 expressed in E. Coli.
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Purification
Affinity Purified
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Conjugation
Unconjugated
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Modification
Unmodified
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Form
Liquid
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Buffer System
Purified antibody in PBS with 0.05% sodium azide
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Storage
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
应用缩写
WB:蛋白质印迹法IHC-P:石蜡切片样本的免疫组织化学法IHC-F:冰冻切片样本的免疫组织化学法ICC/IF:免疫细胞荧光 IP:免疫沉淀法FC:流式细胞术ChIP:染色质免疫沉淀法ELISA:酶联免疫吸附测定
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