anti-Niemann-Pick Disease, Type C1 (NPC1) Antibodies

NPC1 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. Additionally we are shipping NPC1 Proteins (10) and NPC1 Kits (6) and many more products for this protein.

list all antibodies Gene Name GeneID UniProt
NPC1 18145 O35604
NPC1 4864 O15118
NPC1 266732  
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Top anti-NPC1 Antibodies at antibodies-online.com

Showing 10 out of 86 products:

Catalog No. Reactivity Host Conjugate Application Images Quantity Delivery Price Details
Human Mouse Un-conjugated IHC, ELISA, WB 0.1 mg 3 to 4 Days
$430.10
Details
Human Goat Un-conjugated ELISA, WB ABIN1590132 (0.5 µg/mL) staining of HEK293 lysate (35 µg protein in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence. 100 μg 6 to 7 Days
$429.84
Details
Mouse Goat Un-conjugated ELISA   100 μg 6 to 7 Days
$291.53
Details
Human Mouse Un-conjugated Fig. 1. Black line: Control Antigen (100 ng) Fig. 2. Immunohistochemical analysis of paraffin-embedded striated muscle tissues using NPC1 mouse mAb with DAB staining. 100 μg 11 to 16 Days
$469.05
Details
Human Rabbit Un-conjugated ELISA, IHC, IHC (p), WB Anti-NPC1 antibody IHC staining of human adrenal. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody  ABIN959667 concentration 5 ug/ml. Anti-NPC1 antibody IHC staining of human brain, cortex. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody  ABIN959667 concentration 5 ug/ml. 0.05 mg 11 to 14 Days
$484.00
Details
Human Mouse Un-conjugated IF, IHC, IHC (p), IP, WB Human Brain, Cortex (formalin-fixed, paraffin-embedded) stained with NPC1 antibody ABIN462125 followed by biotinylated anti-mouse IgG secondary antibody ABIN481714, alkaline phosphatase-streptavidin and chromogen. Human Colon (formalin-fixed, paraffin-embedded) stained with NPC1 antibody ABIN462125 followed by biotinylated anti-mouse IgG secondary antibody ABIN481714, alkaline phosphatase-streptavidin and chromogen. 50 μg 11 to 14 Days
$727.83
Details
Human Rabbit Un-conjugated ELISA, WB Western blot analysis of Niemann Pick C1 using anti-Niemann Pick C1 antibody .  Electrophoresis was performed on a 5-20% SDS-PAGE gel at 70V (Stacking gel) / 90V (Resolving gel) for 2-3 hours. The sample well of each  Lane was loaded with 50ug of sample under reducing conditions.   Lane 1: rat pancreas tissue lysates, Lane 2: mouse NIH3T3 whole cell lysates.  After Electrophoresis, proteins were transferred to a Nitrocellulose membrane at 150mA for 50-90 minutes. Blocked the membrane with 5% Non-fat Milk/ TBS for 1.5 hour at RT. The membrane was incubated with rabbit anti-Niemann Pick C1 antigen affinity purified polyclonal antibody (Catalog # ) at 0.5 µg/mL overnight at 4°C, then washed with TBS-0.1%Tween 3 times with 5 minutes each and probed with a goat anti-rabbit IgG-HRP secondary antibody at a dilution of 1:10000 for 1.5 hour at RT. The signal is developed using an Enhanced Chemiluminescent detection (ECL) kit (Catalog # EK1002) with Tanon 5200 system. A specific band was detected for Niemann Pick C1 at approximately 170KD. The expected band size for Niemann Pick C1 is at 142KD. 100 μg 4 to 6 Days
$240.00
Details
Mouse Goat Un-conjugated ELISA   100 μg 11 to 14 Days
$507.83
Details
Human Mouse Un-conjugated FACS, IHC, ELISA, WB 100 μg 11 to 16 Days
$469.05
Details
Human Rabbit Un-conjugated IF, IHC, ELISA, WB   100 μg 15 to 19 Days
$527.03
Details

Top referenced anti-NPC1 Antibodies

  1. Chinese Hamster Polyclonal NPC1 Primary Antibody for EM, ICC - ABIN152916 : Infante, Abi-Mosleh, Radhakrishnan, Dale, Brown, Goldstein: Purified NPC1 protein. I. Binding of cholesterol and oxysterols to a 1278-amino acid membrane protein. in The Journal of biological chemistry 2008 (PubMed)
    Show all 32 Pubmed References

  2. Polyclonal NPC1 Primary Antibody for IEM, IHC (fro) - ABIN540364 : Millard, Srivastava, Traub, Schaffer, Ory: Niemann-pick type C1 (NPC1) overexpression alters cellular cholesterol homeostasis. in The Journal of biological chemistry 2001 (PubMed)
    Show all 3 Pubmed References

  3. Human Monoclonal NPC1 Primary Antibody for IHC, ELISA - ABIN1724876 : Morales, Amigo, Balboa, Acuña, Castro, Molina, Miquel, Nervi, Rigotti, Zanlungo: Deficiency of Niemann-Pick C1 protein protects against diet-induced gallstone formation in mice. in Liver international : official journal of the International Association for the Study of the Liver 2010 (PubMed)
    Show all 2 Pubmed References

More Antibodies against NPC1 Interaction Partners

Zebrafish Niemann-Pick Disease, Type C1 (NPC1) interaction partners

  1. this is the first report, showing a role of NPC1 in platelet function and formation but further studies are needed to define how cholesterol storage interferes with these processes

  2. npc1 is required early for proper cell movement and cholesterol localization and later for cell survival

Rabbit Niemann-Pick Disease, Type C1 (NPC1) interaction partners

  1. Availability of assays to measure NPC1 binding to membrne proteins may further the understanding of ways in which oxysterols regulate intracellular lipid transport.

Mouse (Murine) Niemann-Pick Disease, Type C1 (NPC1) interaction partners

  1. Since we had previously shown the beneficial effects of probucol on the somatic phenotype in the Npc1(-/-) mice, we have now studied the effects of combined therapy with HPBCD and probucol on the lung with mostly negative results. Body weight and lung weight for body weight were increased in parallel while inspiratory capacity for body weight was markedly decreased.

  2. We identified CD22 as a marker of dysregulated microglia in Npc1 mutant mice and subsequently demonstrated that elevated cerebrospinal fluid levels of CD22 in NPC1 patients responds to HPbetaCD administration. Collectively, these data provide the first in-depth analysis of microglia function in NPC1 and suggest possible new therapeutic approaches.

  3. extracellular cholesterol concentration in serum under conditions of Npc1 deficiency can influence intracellular cholesterol content/distribution and lysosomal efficacy, triggering the accumulation of toxic APP-cleaved products, eventually leading to cell death.

  4. Mutation in the NPC1 gene is associated with Niemann-Pick type C.

  5. This study shown Microglia can aggravate olfactory dysfunction by mediating neuronal death in the olfactory bulb (OB) of a murine model of Niemann-Pick disease type C1 (NPC1)

  6. High-resolution respirometry analyses revealed that GSH-EE improved oxidative phosphorylation, coupled respiration and maximal electron transfer in cerebellum of Npc1(-/-) mice

  7. Lysosomal oxLDL accumulation within macrophages contributes to murine atherosclerosis. Prevention of oxLDL uptake leads to decreased atherosclerosis in hematopoietic NPC1-deficient Ldlr(-/-) mice

  8. the spleen is significantly enlarged in Npc1(-/-) mice.

  9. Study is the first data to reveal motor and behavioral deficits in early maturity of Npc1+/- mice.

  10. Npc1 gene interacts with a high fat diet to promote weight gain through differential regulation of central energy metabolism pathways.

  11. 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.

  12. Here, we identify lamellar inclusions as the subcellular site of lipid accumulation in neurons, we uncover a vicious cycle of cholesterol synthesis and accretion, which may cause gradual neurodegeneration, and we reveal how beta-cyclodextrin, a potential therapeutic drug, reverts these changes. Our study provides new mechanistic insight in NPC disease and uncovers new targets for therapeutic approaches.

  13. Male NPC1+/- mice had increased fat storage while eating a high-fat diet.

  14. Our data show that: i) HDAC2 levels and activity are increased in NPC neuronal models and in Npc1(-/-) mice; ii) inhibition of c-Abl or c-Abl deficiency prevents the increase of HDAC2 protein levels and activity in NPC neuronal models

  15. activation of the alternative pathway is involved in Niemann-Pick C1 associated pulmonary macrophage accumulation, with low proliferation of these cells balanced by low levels of apoptosis.

  16. This study showed that deleting the Npc1 gene is accompanied by an increase in germ cell apoptosis and compensatory imbalances in the expression of cholesterol enzymatic and transporter factors.

  17. These results show that NPC1 is critical for ebolavirus replication and pathogenesis in animals.

  18. abnormal neuronal expression of matrix metalloproteinase-12 may contribute to axonal degeneration in Niemann-Pick type C disease

  19. There was a transient increase in biliary cholesterol concentration in Npc1(-/-) mice.

  20. data suggest that aberrant expression of Pcdhs is a pathological process accompanied by neurodegeneration in Npc1 mutant mice

Human Niemann-Pick Disease, Type C1 (NPC1) interaction partners

  1. Mutation in the NPC1 gene is associated with Niemann-Pick type C1 disease.

  2. We identified CD22 as a marker of dysregulated microglia in Npc1 mutant mice and subsequently demonstrated that elevated cerebrospinal fluid levels of CD22 in NPC1 patients responds to HPbetaCD administration. Collectively, these data provide the first in-depth analysis of microglia function in NPC1 and suggest possible new therapeutic approaches.

  3. these findings confirm the utility of high-content image-based compound screens of NPC1 patient cells and support extending the approach into larger compound collections.

  4. We also identified LNA ASOs targeting human host factor NPC1 and demonstrated reduced infection by chimeric vesicular stomatitis virus harboring the Ebola glycoprotein, which directly binds to NPC1 for viral infection.

  5. mutant NPC1 degradation is regulated by selective endoplasmic reticulum autophagy and MARCH6-dependent endoplasmic-reticulum-associated degradation

  6. NPC1 gene variations may predispose to common metabolic diseases by modulating steroid hormone synthesis and/or lipid homeostasis.

  7. 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).

  8. These data support the hypothesis that cholesterol is transported through interactions between two or more NPC1 molecules.

  9. Mutation in the NPC1 gene is associated with Niemann-Pick type C.

  10. pronounced alterations in several proteins linked to autophagy and lysosomal catabolism reflecting vesicular transport obstruction and defective lysosomal turnover resulting from NPC1 deficiency, were observed.

  11. Niemann-Pick C1 (NPC1) protein structures suggest mapping of all of the disease-causing mutations for future molecular insights into the pathogenesis of Niemann-Pick type C disease (NPC) disease.

  12. This study demonistrated that heterozygous mutations of NPC1 genes could contribute to dementia plus, at least in a subset of patients.

  13. 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.

  14. Taken together, these studies suggest that Ebola virus requires phosphatidylinositol (3,5) bisphosphate production in cells to promote efficient delivery to NPC1.

  15. 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.

  16. Here we report a crystal structure of a large fragment of human NPC1 at 3.6 A resolution, which reveals internal twofold pseudosymmetry along TM 2-13 and two structurally homologous domains that protrude 60 A into the endosomal lumen, and we propose a model for NPC1 function in cholesterol sensing and transport.

  17. Sequencing of genomic DNA from GM03123 Led to the identification of a mutation in NPC1 GENE, g.41940G>C (c.1947 + 5G>C; rs770321568) (Fig. 1A), with a minor allele frequency of 0.0000082

  18. this case provides support for the V950M variant being sufficient for adult-onset Niemann-Pick type C disease.

  19. We identified major events in NPC1 evolution and revealed and compared orthologs and paralogs of the human NPC1 gene through phylogenetic and protein sequence analyses. We predicted whether an amino acid substitution affects protein function by reducing the organism's fitness.

  20. The mutant NPC1 did not significantly reduce cholesterol accumulation, but approximately 85% of the mutants showed reduced cholesterol accumulation when treated with vorinostat or panobinostat.

NPC1 Antigen Profile

Protein Summary

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.

Gene names and symbols associated with NPC1

  • NPC intracellular cholesterol transporter 1 (NPC1) antibody
  • Niemann-Pick disease, type C1 (npc1) antibody
  • Niemann-Pick C1 protein (LOC579887) antibody
  • NPC intracellular cholesterol transporter 1 (Npc1) antibody
  • A430089E03Rik antibody
  • C85354 antibody
  • Cdig2 antibody
  • D18Ertd139e antibody
  • D18Ertd723e antibody
  • im:7149020 antibody
  • lcsd antibody
  • nmf164 antibody
  • NPC antibody
  • spm antibody
  • wu:fb53a12 antibody
  • wu:fc29a12 antibody

Protein level used designations for NPC1

Niemann-Pick C1 protein , Niemann-Pick type C1 disease protein , Nasopharyngeal carcinoma 1 , Niemann-Pick C1 , Niemann-Pick disease, type C1 , sphingomyelinosis , Niemann-Pick C disease protein

GENE ID SPECIES
403698 Canis lupus familiaris
455338 Pan troglodytes
493693 Felis catus
553330 Danio rerio
579887 Strongylocentrotus purpuratus
100008746 Oryctolagus cuniculus
18145 Mus musculus
4864 Homo sapiens
397591 Sus scrofa
421076 Gallus gallus
266732 Rattus norvegicus
100718604 Cavia porcellus
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