NPC1
Product Sizes
100ug
£787.00
N5379-100UG
About this Product
- SKU:
- N5379
- Application:
- Western Blot, Immunofluorescence
- translate.label.attr.clone:
- 3G214
- Clonality:
- Monoclonal
- Extra Details:
- Niemann-Pick C1 (NPC1) disease is characterized by cholesterol accumulation in lysosomes and aberrant feedback regulation of cellular cholesterol homeostasis. The gene responsible for the disease is NPC1, which encodes a protein with five transmembrane domains. NPC1 protein has homology with the resistance-nodulation-division (RND) family of prokaryotic permeases and may normally function as a transmembrane efflux pump. NPC1 uses a proton motive force to remove accumulated acriflavine from the endosomal/lysosomal (E/L) system. NPC1 can function to transport lipophilic molecules but not cholesterol, out of the E/L system. The fact that NPC1 can transport acriflavine and fatty acids suggest that this permease may have a “multidrug” transport function, part of which is its housekeeping role in cellular cholesterol homeostasis. Expression of NPC1 in E. coli facilitated the transport of acriflavine across the plasma membrane, causing cytosolic accumulation and resulting in transport of oleic acid, but not cholesterol-oleate across the plasma membrane, establishing NPC1 as a eukaryotic member of the RND permease family. Applications: Suitable for use in Immunofluorescence/Immunocytochemistry, Western Blot and ELISA. Other applications have not been tested. Recommended Dilutions: 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.
- Host:
- Mouse
- Immunogen:
- Fragment of human NPC1 conjugated to a carrier protein.
- Isotype:
- IgG1
- Physical State:
- Supplied as a liquid in PBS, pH 7.4, 0.09% sodium azide.
- Purity:
- Purified by Protein A affinity chromatography.
- Shipping Conditions:
- Blue Ice
- Specificity:
- Recognizes human NPC1 protein. Does not cross-react with NPC2.
- Source:
- human
- Storage Conditions:
- -20[o]C
- Supplier:
- United States Biological
- Type:
- Antibodies:Monoclonal Antibody
