214379
NPC1 (Niemann-Pick C1 Protein, NPC)
Size
£652.00
- SKU:
- 214379
- Purity:
- Purified
- Storage Conditions:
- -20°C
- Supplier:
- United States Biological
- Host:
- Mouse
- Immunogen:
- Recombinant protein corresponding to human NPC1 expressed in E. coli.
- Clone:
- 14i194
- Format:
- Supplied as a liquid in PBS, 0.05% sodium azide, 0.5% protein stabilizer.
- 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 ELISA, Western Blot and Immunohistochemistry. Other applications not tested. Recommended Dilution: ELISA: 1:10,000 Western Blot: 1:500-1:2000 Immunohistochemistry: 1:200-1:1000 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. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
- Shipping Conditions:
- Blue Ice