Niemann Pick C1 Rabbit pAb
Cat No.: APA3974
Size：
| Product Name: | Niemann Pick C1 Rabbit pAb |
| Cat No.: | APA3974 |
| source: | Rabbit |
| reactivity: | Human, Mouse, Rat |
| applications: | WB,IHC,ICC/IF,FC |
| clonality: | Polyclonal |
| recommended dilution: | WB: 1:2000 IHC: 1:20 ICC/IF: 1:20 FC: 1:50 |
| format: | Liquid |
| isotype: | IgG |
| immunogen: | A synthetic peptide of human Niemann Pick C1 |
| calculated molecular weight: | 142 kDa |
| observed molecular weight: | 160-180 kDa |
| genbank accession number: | O15118 |
| gene id (ncbi): | 4864 |
| purification method: | Affinity Purification |
| conjugate: | Un-conjugated |
| storage: | Store at -20°C. Supplied in 50nM Tris-Glycine(pH 7.4), 0.15M NaCl, 40%Glycerol, 0.01% sodium azide and 0.05% BSA. Stable for 12 months from date of receipt. |
| synonyms: | NPC |
| category: | Primary Ab |
| concentration: | 0.5mg/ml |
| 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.[provided by RefSeq, Aug 2009] |