Français
English
Antibody Product Overview
| ELK.No | ES14463 |
| Product name | NPC1 rabbit pAb |
| Reactivity | Human; Mouse |
| Applications | WB |
| Other name | |
| Size | 50μL |
| Unit price ($) | 148 |
| Human gene ID | 4864 |
| Human Swiss-Prot | O15118 |
| Source | Rabbit |
| Isotype | IgG |
| Target | NPC1 |
| Fields | >>Lysosome;>>Cholesterol metabolism |
| Gene name | NPC1 |
| Protein name | NPC1 |
| Human gene link | View Human Gene |
| Human Swiss link | View Human Swiss-Prot |
| Mouse gene ID | |
| Mouse gene link | |
| Mouse Swiss-Prot | O35604 |
| Mouse Swiss link | View Mouse Swiss-Prot |
| Rat gene ID | |
| Rat gene link | |
| Rat Swiss-Prot | |
| Rat Swiss link | |
| Immunogen | Synthesized peptide derived from human NPC1 AA range: 1073-1123 |
| Specificity | This antibody detects endogenous levels of NPC1 at Human/Mouse |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Clonality | Polyclonal |
| Dilution | WB 1：500-2000 |
| Purification | The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen. |
| Concentration | 1 mg/ml |
| Storage stability | -20°C/1 year |
| Molecular Weight (Da) | 141kD |
| Observed band (KD) | |
| 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], |
| Function | disease:Defects in NPC1 are the cause of Niemann-Pick disease type C1 (NPC1) [MIM:257220]. NPC1 is an autosomal recessive lipid storage disorder, which affects particularly the brain, liver and spleen, and which is characterized by lysosomal accumulation of low density lipoprotein derived cholesterol. Clinical features include variable hepatosplenomegaly and severe progressive neurological dysfunction such as ataxia, dystonia and dementia. The age of onset can vary from infancy to late adulthood.,disease:Defects in NPC1 are the cause of Niemann-Pick disease type D (NPD) [MIM:257220]; also known as Niemann-Pick disease without sphingomyelinase deficiency, or Nova Scotian type. Because of evidence from biochemical changes, lack of complementation, and linkage mapping to the same chromosome site, NPD and NPC1 are considered to be allelic disorders.,domain:A cysteine-rich N-terminal domain a |
| Subcellular location | Late endosome membrane ; Multi-pass membrane protein . Lysosome membrane ; Multi-pass membrane protein . |
| Expression |
Additional Images
No image
Western blot analysis of lysates from KB cells, primary antibody was diluted at 1:1000, 4°over night
No image
No image
No image
: AO-06-ES14463-50
: 10 Items
Hurry! only 10 items left in stock.
Use collapsible tabs for more detailed information that will help customers make a purchasing decision.
Ex: Shipping and return policies, size guides, and other common questions.
- Paste the label on a flat surface on the package
- Make sure that both 1D and 2D barcodes are clearly visible
- Ensure that the label is smooth and isn’t creased or wrinkled
- Check for any tears, dents, holes or scratches
- Pack your product tightly, with the right size packaging
- Ensure both barcodes are on a flat surface of the package