Niemann Pick C1 Antibody / NPC1
Code | Size | Price |
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NSJ-RQ7282-100UG | 100 ug | £535.00 |
Quantity:
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Overview
Host Type: Rabbit
Antibody Isotype: Rabbit IgG
Antibody Clonality: Polyclonal
Regulatory Status: RUO
Target Species:
- Human
- Mouse
- Rat
Applications:
- Fluorescence-activated cell sorting (FACS)
- Western Blot (WB)
Storage:
After reconstitution, the Niemann Pick C1 antibody can be stored for up to one month at 4°C. For long-term, aliquot and store at -20°C. Avoid repeated freezing and thawing.
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Further Information
Application Details:
Western blot: 0.5-1ug/ml, Flow cytometry: 1-3ug/million cells
Application Note:
Optimal dilution of the Niemann Pick C1 antibody should be determined by the researcher.
Buffer:
Lyophilized from 1X PBS with 2% Trehalose
Description:
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-absorbance 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.
Format:
Antigen affinity purified
Formulation:
0.5mg/ml if reconstituted with 0.2ml sterile DI water
Immunogen:
Amino acids YHTVLQTSADFIDALKKARLI were used as the immunogen for the Niemann Pick C1 antibody.
Limitation:
This Niemann Pick C1 antibody is available for research use only.
Purity:
Antigen affinity purified
Uniprot #:
O15118