Niemann-Pick Disease, Type C2 (NPC2) ELISA Kits

NPC2 encodes a protein containing a lipid recognition domain. Additionally we are shipping NPC2 Antibodies (102) and NPC2 Proteins (18) and many more products for this protein.

list all ELISA KIts Gene Name GeneID UniProt
Anti-Mouse NPC2 NPC2 67963 Q9Z0J0
NPC2 10577 P61916
Anti-Rat NPC2 NPC2 286898  
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Catalog No. Reactivity Sensitivity Range Images Quantity Supplier Delivery Price Details
Human 0.078 ng/mL 0.312-20 ng/mL Typical standard curve 96 Tests Log in to see 15 to 18 Days

More ELISA Kits for NPC2 Interaction Partners

Mouse (Murine) Niemann-Pick Disease, Type C2 (NPC2) interaction partners

  1. these data demonstrate the benefit of a one-time intracisternal administration of AAVrh.10-mNpc2-HA as a life-long treatment for Niemann-Pick type C2 disease

  2. accumulation of free cholesterol in late endosomes/lysosomes of Arf6 knockout mouse embryonic fibroblasts results from mistrafficking of Niemann-Pick type C protein

  3. GARP complex contributes to intracellular cholesterol transport by targeting NPC2 to lysosomes in a CI-MPR-dependent manner.

  4. AAV9-mediated NPC1 delivery significantly promoted Purkinje cell survival, restored locomotor activity and coordination, and increased the lifespan of NPC1(-/-) mice. Our work suggests that AAV-based gene therapy is a promising means to treat NPC disease.

  5. These findings show that NPC2 secreted by premalignant lung tumours suppresses immature macrophage-lineage cell recruitment to the microenvironment in a paracrine manner.

  6. Using the inhibitors of cathepsin enzymatic activity, it was found that cathepsins B and L regulate TNF-alpha production, the expression and secretion of NPC2 protein, and the mRNA levels of the genes involved in cholesterol trafficking in macrophages.

  7. Npc1 and npc2 deficiencies result in pulmonary abnormalities observed in human Niemann-Pick type C disease.

  8. This is the first report demonstrating that GNMT plays an important role in regulating cholesterol homeostasis via interaction with NPC2

  9. NPC1 and NPC2 proteins participate in endosomal/lysosomal processing of both sphingolipids and cholesterol

  10. In liver, absence of either NPC1 or NPC2 resulted in similar alterations in the carbohydrate processing of the lysosomal protease, tripeptidyl peptidase I.

  11. The lack of fibronectin did not interfere with reconstruction of collagen fibril organization in response to liver injury.

  12. NPC2 is a positive regulator of biliary cholesterol secretion via stimulation of ABCG5/G8-mediated cholesterol transport.

  13. Studies showed that processing and export of sterol from the late E/L compartment was quantitatively different in mice lacking LAL, NPC2, or NPC1 function.

  14. NPC2 as a novel intracrine/autocrine factor that controls adipocyte differentiation and function

  15. Results suggest that NPC2 participates in the traffic of ovarian cholesterol required to provide the substrate for steroid synthesis and support follicle maturation, ovulation and luteinization.

  16. NPC2 protein has binding sites with a role in efficient secretion

  17. NPC1 and NPC2 proteins function in concert to facilitate the intracellular transport of lipids from the lysosome to other cellular sites

  18. NPC2 and the majority of sterols secreted from astrocytes are not released together and the secretion of neither sterols nor NPC2 requires NPC1 function

  19. These results suggest that NPC1 and HE1/NPC2 are differentially enriched in astrocytes and neurons, respectively, and that HE1/NPC2 may function in supporting the integrity of the PSD of neurons.

  20. The isolation of late endocytic vesicles from mouse liver, and the metabolism of NPC1 and NPC2 in mice are reported.

Human Niemann-Pick Disease, Type C2 (NPC2) interaction partners

  1. Data show that nuclear factor kappa B subunit 2 (NF-kappaB2) regulates intracellular cholesterol transport by controlling Niemann Pick type C2 protein (NPC2) expression.

  2. Results propose that, depending on the location of the cholesterol ligand, a dynamical interface between the NPC2 and NPC1 N-terminal domain (NTD) proteins exists. Structural features of a particular interface can lower the energy barrier and stabilize the passage of the cholesterol substrate from NPC2 to NPC1(NTD).

  3. Our study demonstrated that NPC2-mediated free cholesterol homeostasis controls hepatic stellate cells proliferation and mitochondrial function.

  4. Niemann-Pick disease type C .E118X NPC2 gene mutation may be prevalent among individuals in Anatolia.

  5. The heterozygous mutations of NPC2 gene could contribute to dementia plus, at least in a subset of patients.

  6. Stopped-Flow Fluorescence Methods for Investigating Intracellular Cholesterol Transport Mechanisms of NPC2 Protein.

  7. Docking of the NPC1-NPC2 complex onto the full-length NPC1 structure reveals a direct cholesterol transfer tunnel between NPC2 and N-terminal domain cholesterol binding pockets, supporting the "hydrophobic hand-off" cholesterol transfer model.

  8. identification of NPC1 and/or NPC2 mutations combined with descriptions of clinical phenotype, will improve our knowledge of pathogenic mutations and our understanding of genotype-phenotype correlations.

  9. 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.

  10. 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.

  11. NEGR1 interacts with NPC2 and increases its protein stability

  12. 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

  13. 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.

  14. Our data suggest an incidence rate for NPC1 and NPC2 of 1/92,104 and 1/2,858,998, respectively. Evaluation of common NPC1 variants, however, suggests that there may be a late-onset NPC1 phenotype with a markedly higher incidence.

  15. Structure of glycosylated NPC1 luminal domain C reveals insights into NPC2 and Ebola virus interactions

  16. hypothesize that, in part, NPC2 rapidly traffics cholesterol between closely appositioned membranes within the multilamellar interior of late endosomal/lysosomal proteins, ultimately effecting cholesterol egress from this compartment

  17. NPC2 may play an important role in negatively regulate cell proliferation.

  18. heterozygous mutations in the NPC1/2 gene might be a risk factor for Alzheimer's disease

  19. Data suggest that in order for the ligand cholesterol to slide from one binding pocket to the other (from NPC2 to NPC1), cholesterol undergoes conformational change/isomerization to accommodate the bent transfer pathway between the 2 binding pockets.

  20. Suggest role for mouse epididymal NPC2 in regulating male fertility.

Cow (Bovine) Niemann-Pick Disease, Type C2 (NPC2) interaction partners

  1. Data suggest ASM (acid sphingomyelinase) activity is regulated by membrane lipids and facilitates cholesterol transfer by NPC2 (Niemann Pick protein type C2); hydrolysis of sphingomyelin by ASM may be crucial for endosomal lipid degradation/sorting.

  2. 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.

  3. bNPC2 has a loosely packed region penetrating from the surface into the hydrophobic core that forms adjacent small cavities and represents an incipient cholesterol-binding site

  4. analysis of sterol binding by NPC2, a lysosomal protein deficient in Niemann-Pick type C2 disease

  5. 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.

  6. 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.

NPC2 Antigen Profile

Antigen Summary

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.

Gene names and symbols associated with NPC2

  • NPC intracellular cholesterol transporter 2 (Npc2) antibody
  • NPC intracellular cholesterol transporter 2 (NPC2) antibody
  • Niemann-Pick disease, type C2 (npc2) antibody
  • 2700012J19Rik antibody
  • AA408070 antibody
  • AU045843 antibody
  • cb292 antibody
  • CE1 antibody
  • EDDM1 antibody
  • EPI-1 antibody
  • HE1 antibody
  • re1 antibody
  • sb:cb292 antibody

Protein level used designations for NPC2

epididymal secretory protein E1 , mE1 , 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 , EPV20 , 16 kDa secretory protein , 16kDa secretory protein , 16.5 kDa secretory protein

67963 Mus musculus
10577 Homo sapiens
286898 Rattus norvegicus
280815 Bos taurus
403920 Canis lupus familiaris
397410 Sus scrofa
450192 Pan troglodytes
282673 Danio rerio
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