146447
NPC1 (Niemann-Pick C1 Protein)
Size
£655.00
- SKU:
- 146447
- Purity:
- Purified by immunoaffinity chromatography.
- Storage Conditions:
- -20°C
- Supplier:
- United States Biological
- Host:
- Rabbit
- Immunogen:
- Synthetic peptide corresponding to 16aa from near the C-terminus of human NPC1.
- Format:
- Supplied as a liquid in PBS, 0.02% sodium azide.
- Extra Details:
- Mutations in the Niemann-Pick disease type C1 (NPC1) gene result in a fatal progressive neurodegenerative disorder characterized by an abnormal sequestration of lipids including cholesterol and glycosphingolipids. The NPC1 protein is 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. NPC1 transports low-density lipoproteins to late endosomal/lysosomal compartments where they are hydrolized and released as free cholesterol. NPC1, in addition to FTO, MC4R, and PTER has recently been shown to be a new risk loci for early-onset and morbid adult obesity in European populations. This anti-NPC1 antibody will not cross-react to NPC2, another gene whose defects also result in Niemann-Pick type C disease. Applications: Suitable for use in ELISA, Western Blot, Immunofluorescence and Immunohistochemistry. Other applications not tested. Recommended Dilution: Western Blot: 1ug/ml Immunohistochemistry (Formalin fixed paraffin embedded): 2.5ug/ml Immunofluorescence: 20ug/ml Optimal dilutions to be determined by the researcher. Positive Control: Human Kidney Tissue Lysate 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