039083
NPC1, ID (NPC1, Niemann-Pick C1 protein)
Size
£696.00
- SKU:
- 039083
- Purity:
- Purified by Protein A and peptide affinity chromatography
- Storage Conditions:
- -20°C
- Supplier:
- United States Biological
- Host:
- Rabbit
- Immunogen:
- KLH-conjugated synthetic peptide mapping to a fragment of residues within amino acids 591-620 in the central region of human NPC1
- Format:
- Supplied as a liquid in PBS, pH 7.2, 0.05% Proclin 300
- Extra Details:
- 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. Applications: Suitable for use in Western Blot, Immunohistochemistry, Immunofluorescence and ELISA. Other applications have not been tested. Recommended Dilutions: Western Blot: 1:1000 Immunofluorescence: 1:10-1:50 Immunohistochemistry (FFPE): 1:10-1:50 Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
- Shipping Conditions:
- Blue Ice