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Monoclonal Anti-NPC1 antibody produced in mouse
产品编号: | 3149407 |
规格: | clone 4H2, purified immunoglobulin, buffered aqueous solution |
包装规格: | 100 μG |
产品类别: | 进口试剂 |
品牌: | Sigma-Aldrich |
优惠价: | 立即咨询 |
产品别名
Monoclonal Anti-NPC1 antibody produced in mouse
Anti-Niemann-Pick disease, type C1
Anti-NPC
基本信息
MDL number | MFCD03455661 |
NACRES | NA.41 |
General description【一般描述】 | 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 |
Immunogen【免疫原】 | NPC1 (AAH63302, 151 a.a. ~ 250 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa. Sequence GFANAMYNACRDVEAPSSNDKALGLLCGKDADACNATNWIEYMFNKDNGQAPFTITPVFSDFPVHGMEPMNNATKGCDESVDEVTAPCSCQDCSIVCGPK |
Physical form【外形】 | Solution in phosphate buffered saline, pH 7.4 |
Legal Information【法律信息】 | GenBank is a registered trademark of United States Department of Health and Human Services |
产品性质
Quality Level【质量水平】 | 100 |
biological source【生物来源】 | mouse |
conjugate【偶联物】 | unconjugated |
antibody form【抗体形式】 | purified immunoglobulin |
antibody product type | primary antibodies |
clone【克隆】 | 4H2, monoclonal |
form【形式】 | buffered aqueous solution |
species reactivity | human |
technique(s) | indirect ELISA: suitable western blot: 1-5 μg/mL |
isotype【同位素/亚型】 | IgG2aκ |
GenBank® accession no. | BC063302 |
UniProt accession no.【UniProt登记号】 | O15118 |
shipped in【运输】 | dry ice |
storage temp.【储存温度】 | −20℃ |
Gene Information | human ... NPC1(4864) |
产品说明
Disclaimer【免责声明】 | Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. |
安全信息
Storage Class Code【储存分类代码】 | 12 - Non Combustible Liquids |
Personal Protective Equipment【个人防护装备】 | Eyeshields, Gloves, multi-purpose combination respirator cartridge (US) |