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Description
Niemann Pick C1 Recombinant Rabbit mAb (S-3160-43)Product Specification Host Rabbit Antigen Niemann Pick C1 Synonyms NPC intracellular cholesterol transporter 1; Niemann Pick C1 protein; NPC1 Immunogen Synthetic Peptide Location Lysosome, Endosome Accession O15118 Clone Number S 3160 43 Antibody Type Recombinant mAb Isotype IgG Application WB Reactivity Hu Positive Sample HEK 293, PC 3, HEK 293 Purification Protein A Concentration 0. 5 mg ml Conjugation Unconjugated Physical Appearance Liquid Storage
Product Specification
| Host | Rabbit |
| Antigen | Niemann Pick C1 |
| Synonyms | NPC intracellular cholesterol transporter 1; Niemann-Pick C1 protein; NPC1 |
| Immunogen | Synthetic Peptide |
| Location | Lysosome, Endosome |
| Accession | O15118 |
| Clone Number | S-3160-43 |
| Antibody Type | Recombinant mAb |
| Isotype | IgG |
| Application | WB |
| Reactivity | Hu |
| Positive Sample | HEK-293, PC-3, HEK-293 |
| Purification | Protein A |
| Concentration | 0.5 mg/ml |
| Conjugation | Unconjugated |
| Physical Appearance | Liquid |
| Storage Buffer | PBS, 40% Glycerol, 0.05% BSA, 0.03% Proclin 300 |
| Stability & Storage | 12 months from date of receipt / reconstitution, -20 °C as supplied |
Dilution
| application | dilution | species |
| WB | 1:1000 | Hu |
Background
Niemann–Pick C1 (NPC1) is a large (~142 kDa) polytopic integral membrane glycoprotein that resides predominantly in the limiting membranes of late endosomes and lysosomes, where it functions as a sterol-sensing shuttle: its N-terminal “cholesterol-binding pocket” captures cholesterol (and other hydrophobic metabolites) from the luminal leaflet, a ring of 13 trans-membrane helices forms a hydrophobic conduit that is gated by cytosolic loop 6, and the C-terminal “NPC2-interacting” domain receives cholesterol from the soluble lysosomal protein NPC2, thereby enabling net export of cholesterol to the cytoplasmic leaflet for redistribution to other organelles; pathogenic mutations in NPC1 block this lipid efflux, causing the fatal neurovisceral disorder Niemann–Pick disease type C1 characterized by lysosomal lipid accumulation, neurodegeneration and premature death.
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