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Item number | Size | Datasheet | Manual | SDS | Delivery time | Quantity | Price |
---|---|---|---|---|---|---|---|
NSJ-RQ4395 | 100 µg | - | - |
3 - 10 business days* |
755.00€
|
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You can also order by e-mail: info@biomol.com
Larger quantity required? Request bulk
You can also order by e-mail: info@biomol.com
Larger quantity required? Request bulk
0.5mg/ml if reconstituted with 0.2ml sterile DI water. Niemann-Pick disease, type C1 (NPC1) is a... more
Product information "Anti-NPC1 / Niemann Pick C1"
0.5mg/ml if reconstituted with 0.2ml sterile DI water. Niemann-Pick disease, type C1 (NPC1) is a membrane protein that mediates intracellular cholesterol trafficking in mammals. In humans it is encoded by the NPC1 gene (chromosome location 18q11). 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. Protein function: Intracellular cholesterol transporter which acts in concert with NPC2 and plays an important role in the egress of cholesterol from the endosomal/lysosomal compartment. Both NPC1 and NPC2 function as the cellular 'tag team duo' (TTD) to catalyze the mobilization of cholesterol within the multivesicular environment of the late endosome (LE) to effect egress through the limiting bilayer of the LE. NPC2 binds unesterified cholesterol that has been released from LDLs in the lumen of the late endosomes/lysosomes and transfers it to the cholesterol-binding pocket of the N-terminal domain of NPC1. Cholesterol binds to NPC1 with the hydroxyl group buried in the binding pocket and is exported from the limiting membrane of late endosomes/ lysosomes to the ER and plasma membrane by an unknown mechanism. Binds oxysterol with higher affinity than cholesterol. May play a role in vesicular trafficking in glia, a process that may be crucial for maintaining the structural and functional integrity of nerve terminals. [The UniProt Consortium]
Keywords: | Anti-Niemann-Pick C1 protein, Anti-NPC intracellular cholesterol transporter 1, NPC1 Antibody / Niemann Pick C1 |
Supplier: | NSJ Bioreagents |
Supplier-Nr: | RQ4395 |
Properties
Application: | WB, ELISA |
Antibody Type: | Polyclonal |
Conjugate: | No |
Host: | Rabbit |
Species reactivity: | human, mouse, rat |
Immunogen: | A human partial recombinant protein corresponding to amino acids A1022-F1278 was used as the immunogen for the NPC1 antibody. |
Format: | Purified |
Database Information
KEGG ID : | K12385 | Matching products |
UniProt ID : | O15118 | Matching products |
Gene ID | GeneID 4864 | Matching products |
Handling & Safety
Storage: | +4°C |
Shipping: | +4°C (International: +4°C) |
Caution
Our products are for laboratory research use only: Not for administration to humans!
Our products are for laboratory research use only: Not for administration to humans!
Information about the product reference will follow.
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