protein

Npc1(mouse) Immunizing Peptide

MBS427309

0.1 mg

190 USD

Peptide

Npc1(mouse) Immunizing Peptide

Npc1

Npc1; Niemann Pick type C1; A430089E03Rik; C85354; D18Ertd139e; D18Ertd723e; lcsd; nmf164; spm; niemann-Pick C1 protein; sphingomyelinosis; Npc1(mouse)

Niemann-Pick C1 protein; Niemann-Pick C1 protein; Niemann-Pick C1 protein; Niemann-Pick type C1

Npc1

Npc1; Npc1; spm; lcsd; C85354; nmf164; D18Ertd139e; D18Ertd723e; A430089E03Rik

NPC1_MOUSE

Mouse

1277

C-TYHTILKTSADYTD

100ug of dried peptide

Shipped at ambient temperature, store at -20 degree C

89242146

NP_032746.2

NM_008720.2

142,885 Da

Lysosome Pathway (99272); Lysosome Pathway (96865)

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, multi-pass; Membrane protein, integral. Cellular Component: endoplasmic reticulum; endosome; extracellular region; Golgi apparatus; integral to membrane; integral to plasma membrane; lipid raft; lysosomal membrane; lysosome; membrane; nuclear envelope; perinuclear region of cytoplasm; plasma membrane; vesicle. Molecular Function: cholesterol binding; hedgehog receptor activity; lipid transporter activity; protein binding. Biological Process: adult walking behavior; autophagy; cholesterol efflux; cholesterol homeostasis; cholesterol metabolic process; cholesterol transport; endocytosis; entry of virus into host cell; lipid metabolic process; lipid raft organization and biogenesis; negative regulation of macroautophagy; protein amino acid glycosylation; response to cadmium ion; response to drug; steroid metabolic process

0.1 mg

190 USD