Niemann-Pick C1 Antibody (NB400-148)
Niemann-Pick C1 Antibody
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anti-Niemann Pick disease type C1 antibody, anti-Niemann Pick disease antibody, anti-Niemann Pick C1 antibody, anti-NPC1 antibody, anti-NPC antibody, anti-Niemann Pick C1 protein precursor antibody, |
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Niemann-Pick C1 Antibody Summary: | | Gene: | NPC1 |
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| Purity: | Affinity purified |
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| Host: | Rabbit |
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| | Specificity: | This is specific for NPC1 protein.
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| Publications: | Antibody has been mentioned in at least 11 Publications. |
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| | Note: Not all species have been tested for usefulness with this product. Only those species listed have been tested. We cannot make any guarantees about additional reactivities which may or may not occur. |
View Additional Niemann-Pick C1 Products Niemann-Pick C1 Antibody Details: | Immunogen: | A synthetic peptide made to the C-terminal region of human NPC1. |
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| Localization: | Late endosome and Lysosome membrane; Single-pass membrane protein. |
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This antibody reacts with human, mouse, and Chinese hamster protein. Other species have not been tested. |
| Uses: | This antibody is useful for Western blot, detecting heterogeneously glycosylated NPC1 protein with prominent bands at 170 and 220 kDa. It has also been tested for immuno-EM (on human protein only). |
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| Dilutions: | Immunofluorescence 1:250, Immunohistochemistry-Paraffin, Immunoprecipitation, Western Blot 1:1000-1:3000 |
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| Unit Size: | 0.1 ml (also available: 0.025 ml (sample size)) |
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| Concentration: | 1.0 mg/ml |
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| Positive Controls: | 1 Positive Controls |
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Packaging: | Storage: | Store at 4 °C. Do not freeze. |
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| Buffer: | PBS |
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| Preservative: | 0.1% Sodium Azide |
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| Limitations: | This product is for research use only and is not approved for use in humans or in clinical diagnosis. Products are guaranteed for 6 months from date of receipt, except for peptides and proteins which are guaranteed for 3 months. |
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Background: Niemann-Pick type C1 (NPC1) is a member of a family of genes encoding membrane-bound proteins containing putative sterol sensing domains. NPC1 protein regulates cholesterol transport from late endosomes-lysosomes to other intracellular compartments. NPC1 overexpression increases the rate of trafficking of low density lipoprotein cholesterol to the endoplasmic reticulum and the rate of delivery of endosomal cholesterol to the plasma membrane.
NPC disease is an inherited neurovisceral lipid storage disorder of unesterified cholesterol accumulation in lysosomes. It is characterized by progressive neural and liver degeneration, resulting from inactivating mutations in NPC1, in most cases.
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Images (2) Reviews (0) Related Products (14) Exploring Niemann-Pick C1 Working with Niemann-Pick C1
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Jump to: Publications, Images, Protocols/Procedures, Ask a Question
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Working with Niemann-Pick C1
Niemann-Pick C1 Antibody Images (2) Western Blot: Niemann Pick C1 antibody [NB400-148] - Detection of human NPC1 in 20 ug of human fibroblast cell lysate using NB 400-148. |  Immunohistochemistry: Niemann Pick C1 antibody [NB400-148] - Brain, Cortex, Neurons and Astrocytes 40x |
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Publications (11)Niemann-Pick C1 Antibody Product Specific:- Lopez ME, Klein AD, Dimbil UJ, Scott MP. Anatomically defined neuron-based rescue of neurodegenerative niemann-pick type C disorder. J. Neurosci. 2011 Mar 23;31(12):4367-4378.[PMID 21430138]
- Charman M, Kennedy BE, Osborne N, et al. MLN64 mediates egress of cholesterol from endosomes to mitochondria in the absence of functional Niemann-Pick Type C1 protein. J Lipid Res. 2010 May;51(5):1023-34. [PMID: 19965586] (WB, Mouse)
more... - Millard E, et al. Niemann-pick type C1 (NPC1) overexpression alters cellular cholesterol homeostasis. J Biol Chem. 2000 Dec 8;275(49):38445-51. [Immunofluorescence, Western blot]
- Gelsthorpe ME, et al. Niemann-Pick type C1 I1061T mutant encodes a functional protein that is selected for endoplasmic reticulum-associated degradation due to protein misfolding. J Biol Chem. 2008 Mar 28;283(13):8229-36. Epub 2008 Jan 23. [Immunoprecipitation]
- Blom T, Bäck N, Mutka AL, et al. FTY720 stimulates 27-hydroxycholesterol production and confers atheroprotective effects in human primary macrophages. Circ Res. 2010 Mar 5;106(4):720-9. [PMID: 20056921]
- Abi-Mosleh L, Infante RE, Radhakrishnan A, et al. Cyclodextrin overcomes deficient lysosome-to-endoplasmic reticulum transport of cholesterol in Niemann-Pick type C cells. Proc Natl Acad Sci U S A. 2009 Nov 17;106(46):19316-21. [PMID: 19884502]
- Frolov A, et al. Cholesterol overload promotes morphogenesis of a Niemann-Pick C (NPC)-like compartment independent of inhibition of NPC1 or HE1/NPC2 function. J Biol Chem. 2001 Dec 7;276(49):46414-21. Epub 2001 Sep 24. [Immunofluorescence]
- Rocha N, Kuijl C, van der Kant R, et al. Cholesterol sensor ORP1L contacts the ER protein VAP to control Rab7-RILP-p150Glued late endosome positioning. J Cell Biol 2009;185(7):1209-1225. [PMID: 19564404]
- Podechard N, Le Ferrec E, Rebillard A, et al. NPC1 repression contributes to lipid accumulation in human macrophages exposed to environmental aryl hydrocarbons. Cardiovasc Res 2009;82(2):361-370. [pubmed: 19131362]
- Burke KT, Colvin PL, Myatt L, et al. Transport of maternal cholesterol to the fetus is affected by maternal plasma cholesterol concentrations in the golden Syrian hamster. J Lipid Res. 2009 Jun;50(6):1146-55. Epub 2009 Jan 3.
- Infante, R. E., et al. Purified NPC1 protein. I. Binding of cholesterol and oxysterols to a 1278-amino acid membrane protein. J Biol Chem. 2008 Jan 11;283(2):1052-63. Epub 2007 Nov 6.
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Related ProductsPositive Controls| Name | Catalog# | Reactivity | Applications | Images | Publications | Reviews | |
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Other Positive Controls- Human fibroblast cell lysate
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Peptides and ProteinsPrimary Antibodies (11)| Name | Catalog# | Reactivity | Applications | Images | Publications | Reviews | |
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| Niemann-Pick C1 Antibody (EPR5209) | NBP1-95817 | Hu, Mu, Rt | ICC, IHC-P, WB | (2) | | | | | Niemann-Pick C1 Antibody | PAB12093 | Ha, Hu, Mu | IF, IHC-P, IP, WB | | | | | | Niemann-Pick C1 Antibody (4H2) | H00004864-M02 | Hu | ELISA, WB | (1) | (1) | | | | Niemann-Pick C1 Antibody | PAB16750 | Hu | ELISA, IHC-P, WB | | | | | | Niemann-Pick C1 Antibody | PAB18649 | Mu | ELISA | | | | | | ... and 6 more. See All » |
RNAi (1)Secondary Antibodies| Name | Catalog# | Reactivity | Applications | Images | Publications | Reviews | |
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| Rabbit IgG Antibody [HRP] | NB730-H | Rb | ELISA, ICC, IHC-P, WB | | (5) | | | | Rabbit IgG Antibody [FITC] | NB730-F | Rb | ICC, IHC-P | | (1) | | | | Rabbit IgG Antibody [Biotin] | NB730-B | Rb | ELISA, ICC, IHC-P, WB | | | | | | Rabbit IgG, H/L Chains Antibody [DyLight 488] | NBP1-72944 | Rb | FLISA, IF, WB | (3) | | | | | Rabbit IgG Antibody [Alkaline Phosphatase] | NB730-AP | Rb | ELISA, ICC, IHC-P, WB | | (1) | | | | See All » |
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