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NPC2 encodes a protein containing a lipid recognition domain. Zusätzlich bieten wir Ihnen NPC2 Antikörper (85) und NPC2 Kits (7) und viele weitere Produktgruppen zu diesem Protein an.
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AAV9-mediated NPC1 (zeige NPC1 Proteine) delivery significantly promoted Purkinje cell survival, restored locomotor activity and coordination, and increased the lifespan of NPC1 (zeige NPC1 Proteine)(-/-) mice. Our work suggests that AAV-based gene therapy is a promising means to treat NPC (zeige NPC1 Proteine) disease.
These findings show that NPC2 secreted by premalignant lung tumours suppresses immature macrophage-lineage cell recruitment to the microenvironment in a paracrine manner.
Using the inhibitors of cathepsin enzymatic activity, it was found that cathepsins B and L regulate TNF-alpha (zeige TNF Proteine) production, the expression and secretion of NPC2 protein, and the mRNA levels of the genes involved in cholesterol trafficking in macrophages.
Npc1 (zeige NPC1 Proteine) and npc2 deficiencies result in pulmonary abnormalities observed in human Niemann-Pick type C disease.
This is the first report demonstrating that GNMT (zeige GNMT Proteine) plays an important role in regulating cholesterol homeostasis via interaction with NPC2
NPC1 (zeige NPC1 Proteine) and NPC2 proteins participate in endosomal/lysosomal processing of both sphingolipids and cholesterol
In liver, absence of either NPC1 (zeige NPC1 Proteine) or NPC2 resulted in similar alterations in the carbohydrate processing of the lysosomal protease, tripeptidyl peptidase I (zeige TPP1 Proteine).
The lack of fibronectin (zeige FN1 Proteine) did not interfere with reconstruction of collagen fibril organization in response to liver injury.
NPC2 is a positive regulator of biliary cholesterol secretion via stimulation of ABCG5 (zeige ABCG5 Proteine)/G8-mediated cholesterol transport.
Studies showed that processing and export of sterol from the late E/L compartment was quantitatively different in mice lacking LAL (zeige LIPA Proteine), NPC2, or NPC1 (zeige NPC1 Proteine) function.
The heterozygous mutations of NPC2 gene could contribute to dementia plus, at least in a subset of patients.
Stopped-Flow Fluorescence Methods for Investigating Intracellular Cholesterol Transport Mechanisms of NPC2 Protein.
Docking of the NPC1 (zeige NPC1 Proteine)-NPC2 complex onto the full-length NPC1 (zeige NPC1 Proteine) structure reveals a direct cholesterol transfer tunnel between NPC2 and N-terminal domain cholesterol binding pockets, supporting the "hydrophobic hand-off" cholesterol transfer model.
identification of NPC1 (zeige NPC1 Proteine) and/or NPC2 mutations combined with descriptions of clinical phenotype, will improve our knowledge of pathogenic mutations and our understanding of genotype-phenotype correlations.
Overall, we provide a mechanism by which npc2-mediated cholesterol transport is controlled by the membrane composition and how npc2-lipid interactions can regulate the transport rate.
Our results suggest that NPC2 is in a mitochondrially associated autophagosome and plays an important role in regulating mitophagy, mitochondrial quality control, and mitochondrial function.
NEGR1 (zeige NEGR1 Proteine) interacts with NPC2 and increases its protein stability
suggest a general mechanism for NPC2 mediated sterol transfer, in which Phe66, Val96, and Tyr100 act as reversible gate keepers. These residues stabilize the sterol in the binding pose via pi-pi stacking but move transiently apart during sterol release
Study demonstrates that Niemann-Pick type C disease can present in early years of life with pulmonary complications like alveolar proteinosis and hepatosplenomegaly or hepatomegaly due to mutation in NPC2 gene.
Our data suggest an incidence rate for NPC1 (zeige NPC1 Proteine) and NPC2 of 1/92,104 and 1/2,858,998, respectively. Evaluation of common NPC1 (zeige NPC1 Proteine) variants, however, suggests that there may be a late-onset NPC1 (zeige NPC1 Proteine) phenotype with a markedly higher incidence.
Data suggest ASM (acid sphingomyelinase (zeige SMPD1 Proteine)) activity is regulated by membrane lipids and facilitates cholesterol transfer by NPC2 (Niemann Pick protein type C2); hydrolysis of sphingomyelin by ASM (zeige SMPD1 Proteine) may be crucial for endosomal lipid degradation/sorting.
Data show that the effects of the lipids on cholesterol transfer mediated by NPC2 were similar to their effect on membrane fusion induced by NPC2 and saposin-C.
analysis of sterol binding by NPC2, a lysosomal protein deficient in Niemann-Pick type C2 disease
The seminal plasma protein, Niemann-Pick C2 protein, is involved in cholesterol and GM1 depletion within detergent-resistant membrane, then leading to membrane redistribution of P25b that occurs in a very rapid and capacitation-independent manner.
NPC2 plays an important role in endo/lysosomal cholesterol trafficking by markedly accelerating the rates of cholesterol transport. Rates of sterol transfer from and between membranes were increased by as much as 2 orders of magnitude by NPC2.
This gene encodes a protein containing a lipid recognition domain. The encoded protein may function in regulating the transport of cholesterol through the late endosomal/lysosomal system. Mutations in this gene have been associated with Niemann-Pick disease, type C2 and frontal lobe atrophy.
epididymal secretory protein E1
, niemann Pick type C2 protein homolog
, Niemann-Pick disease type C2 protein
, epididymal protein 1
, human epididymis-specific protein 1
, tissue-specific secretory protein
, Niemann Pick type C2
, epididymal secretory protein 1
, 16 kDa secretory protein
, 16kDa secretory protein
, 16.5 kDa secretory protein