克隆類型 : |
Rabbit Polyclonal Antibody |
亞 型 : |
Rabbit Ig |
免 疫 原 : |
KLH-conjugated synthetic peptide encompassing a sequence within human NPC1. |
產(chǎn)品描述 : |
Mutations in the Niemann-Pick disease type C1 (NPC1) gene result in a fatal progressive neurodegenerative disorder characterized by an abnormal sequestration of lipids including cholesterol and glycosphingolipids. The NPC1 protein is 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. NPC1 transports low-density lipoproteins to late endosomal/lysosomal compartments where they are hydrolized and released as free cholesterol. NPC1, in addition to FTO, MC4R, and PTER has recently been shown to be a new risk loci for early-onset and morbid adult obesity in European populations. This anti-NPC1 antibody will not cross-react to NPC2, another gene whose defects also result in Niemann-Pick type C disease. |
適用物種 : |
H |
應(yīng)用范圍 : |
E, WB, IHC |
應(yīng)用說明 : |
E: 1:500-1:1,000; WB: 1:100-1:500; IHC: 1:100-1:500 |
別 名 : |
NPC1; Niemann-Pick disease type C1; NPC |
形 態(tài) : |
Each vial contains 0.1 ml IgG in PBS pH 7.4 with 0.02% sodium azide. Antibody was purified by immunogen affinity chromatography. |
保存條件 : |
Store at -20°C. Minimize freeze-thaw cycles. Product is guaranteed one year from the date of shipment. |
產(chǎn)品鏈接 : |
http://www.abbiotec.com/antibodies/npc1-antibody |
標(biāo) 記 物 : |
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產(chǎn)品說明 : |
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