Mouse Npc1 (NPC intracellular cholesterol transporter 1) ELISA Kit | Npc1 elisa kit
Mouse Npc1 (NPC intracellular cholesterol transporter 1) ELISA Kit
NCBI and Uniprot Product Information
Uniprot Description
NPC1: 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. Defects in NPC1 are the cause of Niemann-Pick disease type C1 (NPC1). A lysosomal storage disorder that affects the viscera and the central nervous system. It is due to defective intracellular processing and transport of low-density lipoprotein derived cholesterol. It causes accumulation of cholesterol in lysosomes, with delayed induction of cholesterol homeostatic reactions. Niemann-Pick disease type C1 has a highly variable clinical phenotype. Clinical features include variable hepatosplenomegaly and severe progressive neurological dysfunction such as ataxia, dystonia and dementia. The age of onset can vary from infancy to late adulthood. An allelic variant of Niemann-Pick disease type C1 is found in people with Nova Scotia ancestry. Patients with the Nova Scotian clinical variant are less severely affected. Belongs to the patched family.
Protein type: Membrane protein, integral; Membrane protein, multi-pass
Cellular Component: Golgi apparatus; lysosomal membrane; endoplasmic reticulum; lysosome; integral to plasma membrane; integral to membrane; extracellular region; nuclear envelope; lipid raft; membrane; perinuclear region of cytoplasm; plasma membrane; endosome; vesicle
Molecular Function: protein binding; hedgehog receptor activity; cholesterol binding
Biological Process: response to drug; steroid metabolic process; cholesterol metabolic process; cholesterol transport; protein amino acid glycosylation; endocytosis; cholesterol efflux; adult walking behavior; negative regulation of macroautophagy; cholesterol homeostasis; response to cadmium ion; autophagy; lipid metabolic process; lipid raft organization and biogenesis
Research Articles on Npc1
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Product Notes
The Mouse Npc1 npc1 (Catalog #AAA7612396) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA7612396 ELISA Kit recognizes Mouse Npc1. It is sometimes possible for the material contained within the vial of "Npc1 (NPC intracellular cholesterol transporter 1), ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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