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

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. Zusätzlich bieten wir Ihnen NPC1 Proteine (10) und NPC1 Kits (5) und viele weitere Produktgruppen zu diesem Protein an.

Alle Antikörper anzeigen Gen GeneID UniProt
NPC1 18145 O35604
NPC1 4864 O15118
NPC1 266732  
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Top anti-NPC1 Antikörper auf antikoerper-online.de

Showing 10 out of 73 products:

Katalog Nr. Reaktivität Wirt Konjugat Applikation Bilder Menge Anbieter Lieferzeit Preis Details
Chinese Hamster Kaninchen Unkonjugiert 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 Anmelden zum Anzeigen 7 bis 9 Tage
$447.56
Details
Human Maus Unkonjugiert IHC, ELISA, WB 100 μg Anmelden zum Anzeigen 2 bis 3 Tage
$430.10
Details
Human Maus Unkonjugiert ELISA, WB Detection limit for recombinant GST tagged NPC1 is approximately 0.03 ng/mL as a capture antibody. 0.1 mg Anmelden zum Anzeigen 8 bis 11 Tage
$450.00
Details
Human Kaninchen Unkonjugiert 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 Anmelden zum Anzeigen 10 bis 11 Tage
$324.50
Details
Human Kaninchen Unkonjugiert 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 Anmelden zum Anzeigen 7 bis 9 Tage
$451.00
Details
Human Maus PE FACS 100 Tests Anmelden zum Anzeigen 10 bis 12 Tage
$282.64
Details
Maus Ziege Unkonjugiert ELISA   100 μg Anmelden zum Anzeigen 7 bis 9 Tage
$507.83
Details
Human Kaninchen Unkonjugiert EIA, IHC (p), WB   0.1 mg Anmelden zum Anzeigen 12 bis 15 Tage
$375.38
Details
Human Ziege Unkonjugiert ELISA, WB   0.1 mg Anmelden zum Anzeigen 2 bis 3 Tage
$446.88
Details
Human Maus Unkonjugiert 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 Anmelden zum Anzeigen 7 bis 9 Tage
$676.50
Details

NPC1 Antikörper nach Reaktivität, Anwendung, Klonalität and Konjugat

Attribute Applikationen Wirt Klonalität Konjugat
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Am meisten referenzierte anti-NPC1 Antikörper

  1. Chinese Hamster Polyclonal NPC1 Primary Antibody für 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. Human Monoclonal NPC1 Primary Antibody für 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

  3. Human Monoclonal NPC1 Primary Antibody für 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. in Stem cell reports 2014 (PubMed)

Weitere Antikörper gegen NPC1 Interaktionspartner

Zebrafish Niemann-Pick Disease, Type C1 (NPC1) Interaktionspartner

  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) Interaktionspartner

  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) Interaktionspartner

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

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

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

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

  5. 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.

  6. 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.

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

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

  9. 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.

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

Human Niemann-Pick Disease, Type C1 (NPC1) Interaktionspartner

  1. 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.

  2. 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.

  3. knockdown of TMEM97 (zeige TMEM97 Antikörper) also increases levels of residual NPC1 in NPC1-mutant patient fibroblasts and reduces cholesterol storage in an NPC1-dependent manner. Our findings propose TMEM97 (zeige TMEM97 Antikörper) 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).

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

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

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

  7. Our data suggest an incidence rate for NPC1 and NPC2 (zeige NPC2 Antikörper) 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.

  8. 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 (zeige CTSD Antikörper) activity.

  9. Results identified six novel mutations (PKHD1 (zeige PKHD1 Antikörper): p.Thr777Met, p.Tyr2260Cys; ABCB11 (zeige ABCB11 Antikörper): p.Val1112Phe, c.611+1G > A, p.Gly628Trpfs*3 and NPC1: p.Glu391Lys) for the diagnostic of inherited infantile cholestatic disorders.

  10. NPC1 mutations are substantially enriched in unexplained early onset ataxia, making it high risk group for Niemann-Pick disease type C.

NPC1 Antigen-Profil

Protein Überblick

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.

Genbezeichner und Symbole assoziert mit NPC1

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

Bezeichner auf Proteinebene für 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 SPEZIES
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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