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 (5) 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 70 products:

Catalog No. Reactivity Host Conjugate Application Images Quantity Supplier Delivery Price Details
Human Mouse Un-conjugated ELISA, WB Detection limit for recombinant GST tagged NPC1 is approximately 0.03 ng/mL as a capture antibody. 0.1 mg Log in to see 8 to 11 Days
$450.00
Details
Human Mouse Un-conjugated IHC, ELISA, WB 0.1 mg Log in to see 2 to 3 Days
$430.10
Details
Chinese Hamster Rabbit Un-conjugated 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 Log in to see 7 to 9 Days
$447.56
Details
Human Rabbit Un-conjugated 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 Log in to see 10 to 11 Days
$324.50
Details
Human Mouse PE FACS 100 Tests Log in to see 10 to 12 Days
$282.64
Details
Human Rabbit Un-conjugated 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 Log in to see 7 to 9 Days
$451.00
Details
Human Rabbit Un-conjugated EIA, IHC (p), WB   0.1 mg Log in to see 12 to 15 Days
$375.38
Details
Human Goat Un-conjugated ELISA, WB   0.1 mg Log in to see 2 to 3 Days
$446.88
Details
Human Mouse Un-conjugated 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 Log in to see 7 to 9 Days
$595.83
Details
Mouse Goat Un-conjugated ELISA   100 μg Log in to see 7 to 9 Days
$419.83
Details

NPC1 Antibodies by Reactivity, Application, Clonality and Conjugate

Attributes Applications Host Clonality Conjugate
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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 references for 152916

  2. Human Monoclonal NPC1 Primary Antibody for FACS - ABIN2663864 : Robertson, Gip, Schaffer: Neural stem cell engineering: directed differentiation of adult and embryonic stem cells into neurons. in Frontiers in bioscience : a journal and virtual library 2007 (PubMed)
    Show all 3 references for 2663864

  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 references for 1724876

  4. Human Monoclonal NPC1 Primary Antibody for 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)

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. 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.

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

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

  4. 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.

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

  6. abnormal neuronal expression of matrix metalloproteinase-12 (show MMP12 Antibodies) may contribute to axonal degeneration in Niemann-Pick type C disease

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

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

  9. This study deministrated that lack of NPC1 in either neurons or glial cells did not affect the excitability of Purkinje cells, the formation of dendrites or their excitatory synaptic activity.

  10. Hearing loss is an early consequence of Npc1 gene deletion in the mouse model of Niemann-Pick disease, type C.

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

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

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

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

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

  5. Our data suggest an incidence rate for NPC1 and NPC2 (show NPC2 Antibodies) 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.

  6. 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 (show CTSD Antibodies) activity.

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

  8. NPC1 mutations are substantially enriched in unexplained early onset ataxia (show USP14 Antibodies), making it high risk group for Niemann-Pick disease type C.

  9. these results clearly demonstrated that the over-expression of NPC1 with a defective function in an imatinib-resistant Ph+ acute lymphoblastic leukemia cell line

  10. Structure of glycosylated NPC1 luminal domain C reveals insights into NPC2 (show NPC2 Antibodies) and Ebola virus interactions

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

  • Niemann-Pick disease, type C1 (NPC1) antibody
  • Niemann-Pick disease, type C1 (npc1) antibody
  • Niemann Pick type C1 (Npc1) antibody
  • Niemann-Pick disease, type C1 (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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