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| Catalog Code: | NB400-148 |
| Product Name: | Niemann-Pick C1 Antibody |
| Product Description: | Rabbit Polyclonal anti-Niemann-Pick C1 |
| Clonality: | Polyclonal |
| ListPrice: | 285 |
| Gene ID: | 4864 |
| Concentration: | 1.3 mg/ml |
| Immunogen: | A synthetic peptide made to the C-terminal region of human NPC1. |
| Specificity: | This antibody is specific for NPC1 protein. |
| Cross Reactivity: | This antibody reacts with human, mouse, and hamster protein. Other species have not been tested. |
| Packaging: | 0.1 ml Affinity purified Rabbit antisera. |
| 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). |
| Localization: | Late endosome and Lysosome membrane; Single-pass membrane protein. |
| 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. |
| Storage: | Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze thaw cycles. |
| Purity: | Affinity purified |
| Host Name: | Rabbit |
| Buffer: | PBS |
| Application Summary: | immunohistochemistry , immunofluorescence 1:250, immunoprecipitation , Western Blot 1:1000-1:3000 |
| Species Summary: | Ha, Hu, Mu, , |
| Alternate Names: | anti-NPC antibody; anti-NPC1 antibody; anti-Niemann Pick C1 antibody; anti-Niemann Pick C1 protein precursor antibody; anti-Niemann Pick disease antibody; anti-NPC antibody; anti-NPC1 antibody; anti-Niemann Pick C1 antibody; anti-Niemann Pick C1 protein precursor antibody; anti-Niemann Pick disease antibody |
| Package Size: | 0.1 ml |
| Preservative: | 0.1% sodium azide |
| Product References: | 1. Millard E, et al. Niemann-Pick type C1 (NPC1) overexpression alters cellular cholesterol homeostasis. JBC. 275(49): 38445-51. 2000 [Immunofluorescence and Western blot] 2. 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. JBC. 276: 46414-21. 2001 [Immunofluorescence] |
| Novus References: | 1. Infante, R., et al. Purified NPC1 protein: I. Binding of cholesterol and oxysterols to a 1278-amino acid membrane protein. J. Biol. Chem published 6 November 2007, 10.1074/jbc.M707943200. 2. Infante, R. E., et al. Purified NPC1 Protein: I. BINDING OF CHOLESTEROL AND OXYSTEROLS TO A 1278-AMINO ACID MEMBRANE PROTEIN. J. Biol. Chem. Jan 2008; 283: 1052 - 1063. |
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