深圳欣博盛生物科技有限公司 a1

Niemann Pick C1 Antibody / NPC1
货号:RQ7282 规格:100 ug 目录价:¥6488.05
产品详情
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产品名称:
Niemann Pick C1 Antibody / NPC1
产品描述:
Niemann-Pick disease, type C1 (NPC1) is a membrane protein that mediates intracellular cholesterol trafficking in mammals. In humans it is encoded by the NPC1 gene (chromosome location 18q11). 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-absorbance 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.
反应种属:
Human, Mouse, Rat
宿主来源:
Rabbit
实验应用:
WB, FACS
同种型:
Rabbit IgG
免疫原:
Amino acids YHTVLQTSADFIDALKKARLI were used as the immunogen for the Niemann Pick C1 antibody.
克隆性:
Polyclonal (rabbit origin)
纯化方式:
Antigen affinity purified
保存温度:
After reconstitution, the Niemann Pick C1 antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.
产品形式:
Antigen affinity purified
存储溶液:
0.5mg/ml if reconstituted with 0.2ml sterile DI water
产地:
美国
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