NPC1 rabbit pAb

NPC1 rabbit pAb

• Description: 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. [provided by RefSeq, Aug 2009],
• Gene ID: 4864
• UniProt: O15118
• Cellular Locus: Late endosome membrane ; Multi-pass membrane protein. Lysosome membrane ; Multi-pass membrane protein.
• Host: Rabbit
• Species Reactivity: Human, Mouse
• Immunogen: Synthesized peptide derived from human NPC1 AA range: 1073-1123
• Clonality: Polyclonal
• Validated Applications: WB
• Stability: 1 year
• Concentration: 1 mg/mL
• Dilution: WB 1：500-2000
• Storage Conditions: PBS with 0.02% sodium azide and 50% glycerol pH 7.4. Store at -20°C. Avoid repeated freeze-thaw cycles.