|Application ||WB, IF, IHC-P, E|
|Calculated MW||142167 Da|
|Antigen Region||591-620 aa|
|Other Names||Niemann-Pick C1 protein, NPC1|
|Target/Specificity||This NPC1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 591-620 amino acids from the Central region of human NPC1.|
|Format||Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.|
|Storage||Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||NPC1 Antibody (Center) is for research use only and not for use in diagnostic or therapeutic procedures.|
|Function||Intracellular cholesterol transporter which acts in concert with NPC2 and plays an important role in the egress of cholesterol from the endosomal/lysosomal compartment. Both NPC1 and NPC2 function as the cellular 'tag team duo' (TTD) to catalyze the mobilization of cholesterol within the multivesicular environment of the late endosome (LE) to effect egress through the limiting bilayer of the LE. NPC2 binds unesterified cholesterol that has been released from LDLs in the lumen of the late endosomes/lysosomes and transfers it to the cholesterol-binding pocket of the N-terminal domain of NPC1. Cholesterol binds to NPC1 with the hydroxyl group buried in the binding pocket and is exported from the limiting membrane of late endosomes/ lysosomes to the ER and plasma membrane by an unknown mechanism. Binds oxysterol with higher affinity than cholesterol. May play a role in vesicular trafficking in glia, a process that may be crucial for maintaining the structural and functional integrity of nerve terminals.|
|Cellular Location||Late endosome membrane; Multi-pass membrane protein. Lysosome membrane; Multi-pass membrane protein|
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Provided below are standard protocols that you may find useful for product applications.
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.
Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010)
Fontaine-Bisson, B., et al. Diabetologia 53(10):2155-2162(2010)
Kagedal, K., et al. Biochim. Biophys. Acta 1801(8):831-838(2010)
Rodriguez-Rodriguez, E., et al. J. Alzheimers Dis. 21(2):619-625(2010)
Ma, W., et al. BMC Med. Genet. 11, 149 (2010) :
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