Titration of the NPC1 antibody may be required due to differences in protocols and secondary/substrate sensitivity.\. Western blot: 1:1000,Immunofluorescence: 1:10-1:50,IHC (Paraffin): 1:10-1:50
限制
仅限研究用
状态
Liquid
缓冲液
In 1X PBS, pH 7.4, with 0.09 % 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
-20 °C
储存方法
Aliquot the NPC1 antibody and store frozen at -20°C or colder. Avoid repeated freeze-thaw cycles.
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.