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

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. De plus, nous expédions NPC1 Protéines (10) et NPC1 Kits (5) et beaucoup plus de produits pour cette protéine.

afficher tous les anticorps Gène GeneID UniProt
NPC1 18145 O35604
NPC1 4864 O15118
NPC1 266732  
Comment commander chez anticorps-enligne
  • +1 877 302 8632
  • +1 888 205 9894 (toll-free)
  • Commandez enligne
  • orders@anticorps-enligne.fr

Top anti-NPC1 Anticorps sur anticorps-enligne.fr

Showing 10 out of 73 products:

Catalogue No. Reactivité Hôte Conjugué Application Images Quantité Fournisseur Livraison Prix Détails
Chinese Hamster Lapin Inconjugué EM, ICC, IF, IHC, IHC (p), WB Detection of human NPC1 in 20 ug of human fibroblast cell lysate using ABIN152916. Brain, Cortex, Neurons and Astrocytes 40x 0.1 mL Connectez-vous pour afficher 7 to 9 Days
$447.56
Détails
Humain Souris Inconjugué IHC, ELISA, WB 100 μg Connectez-vous pour afficher 2 to 3 Days
$430.10
Détails
Humain Souris Inconjugué ELISA, WB Detection limit for recombinant GST tagged NPC1 is approximately 0.03 ng/mL as a capture antibody. 0.1 mg Connectez-vous pour afficher 8 to 11 Days
$450.00
Détails
Humain Lapin Inconjugué IF, IHC (p), WB NPC1 Antibody (Center)  western blot analysis in NCI-H460 cell line lysates (35ug/lane).This demonstrates the NPC1 antibody detected the NPC1 protein (arrow). Confocal immunofluorescent analysis of NPC1 Antibody (Center)  with 293 cell followed by Alexa Fluor 488-conjugated goat anti-rabbit lgG (green). DAPI was used to stain the cell nuclear (blue). 400 μL Connectez-vous pour afficher 10 to 11 Days
$324.50
Détails
Humain Lapin Inconjugué IHC (p), ELISA, 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. 50 μg Connectez-vous pour afficher 7 to 9 Days
$451.00
Détails
Humain Souris PE FACS 100 Tests Connectez-vous pour afficher 10 to 12 Days
$282.64
Détails
Souris Chèvre Inconjugué ELISA   100 μg Connectez-vous pour afficher 7 to 9 Days
$507.83
Détails
Humain Lapin Inconjugué EIA, IHC (p), WB   0.1 mg Connectez-vous pour afficher 12 to 15 Days
$375.38
Détails
Humain Chèvre Inconjugué ELISA, WB   0.1 mg Connectez-vous pour afficher 2 to 3 Days
$446.88
Détails
Humain Souris Inconjugué IF, 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 Connectez-vous pour afficher 7 to 9 Days
$676.50
Détails

NPC1 Anticorps par réactivité, application, clonalité et conjugué

Attributs Application Hôte Clonalité Conjugué
Mouse (Murine) ,
,

, , , , , ,
Human , , ,
, ,
,
, , , , , ,
Rat (Rattus) ,


, ,

anti-NPC1 Anticorps mieux référencés

  1. Chinese Hamster Polyclonal NPC1 Primary Antibody pour 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. dans The Journal of biological chemistry 2008 (PubMed)
    Show all 32 Pubmed References

  2. Human Monoclonal NPC1 Primary Antibody pour 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. dans Liver international : official journal of the International Association for the Study of the Liver 2010 (PubMed)
    Show all 2 Pubmed References

  3. Human Monoclonal NPC1 Primary Antibody pour ELISA, WB - ABIN396466 : Maetzel, Sarkar, Wang, Abi-Mosleh, Xu, Cheng, Gao, Mitalipova, Jaenisch: Genetic and chemical correction of cholesterol accumulation and impaired autophagy in hepatic and neural cells derived from Niemann-Pick Type C patient-specific iPS cells. dans Stem cell reports 2014 (PubMed)

Plus d’anticorps contre NPC1 partenaires d’interaction

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

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

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

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

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

  6. 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.

  7. 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.

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

  9. Our data show that: i) HDAC2 (Montrer HDAC2 Anticorps) levels and activity are increased in NPC neuronal models and in Npc1(-/-) mice; ii) inhibition of c-Abl (Montrer ABL1 Anticorps) or c-Abl (Montrer ABL1 Anticorps) deficiency prevents the increase of HDAC2 (Montrer HDAC2 Anticorps) protein levels and activity in NPC neuronal models

  10. 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.

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

  1. 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 (Montrer TPM2 Anticorps)-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.

  2. 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

  3. 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.

  4. 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.

  5. knockdown of TMEM97 (Montrer TMEM97 Anticorps) also increases levels of residual NPC1 in NPC1-mutant patient fibroblasts and reduces cholesterol storage in an NPC1-dependent manner. Our findings propose TMEM97 (Montrer TMEM97 Anticorps) inhibition as a novel strategy to increase residual NPC1 levels in cells and a potential therapeutic target for Niemann-Pick type C disease (NP-C).

  6. Rare loss-of-function NPC1 mutations were identified as being associated with human adiposity with a high penetrance in a Chinese population.

  7. Furthermore saturation and intracellular distribution of alpha-Toc (Montrer RHBDF2 Anticorps) seem to be strongly dependent on the availability of this vitamin as well as on the presence of the lysosomal protein NPC1

  8. Two mutations were identified in the NPC1 gene, one of which was novel and its pathogenetic nature was unknown

  9. Our data suggest an incidence rate for NPC1 and NPC2 (Montrer NPC2 Anticorps) 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.

  10. Fibroblasts from Niemann-Pick type C (NPC) disease patients with low levels of NPC1 protein have high amounts of procathepsin D but reduced quantities of the mature protein, thus showing a diminished cathepsin D (Montrer CTSD Anticorps) activity.

NPC1 profil antigène

Profil protéine

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

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
Fournisseurs de qualité sélectionnés pour anti-NPC1 (NPC1) Anticorps
Avez-vous cherché autre chose?