Specifications
- HOST SPECIES: Rabbit
- SPECIES REACTIVITY: Human, Mouse
- HOMOLOGY: Predicted species reactivity based on immunogen sequence: Pig: (87%)
- IMMUNOGEN: NPC1 antibody was raised against a 16 amino acid synthetic peptide from near the carboxy terminus of human NPC1.
The immunogen is located within the last 50 amino acids of NPC1. - CONJUGATE: Unconjugated
- TESTED APPLICATIONS: ELISA, IF, IHC-P, WB
- APPLICATION NOTE: NPC1 antibody can be used for detection of NPC1 by Western blot at 1 μg/mL. Antibody can also be used for immunohistochemistry starting at 2.5 μg/mL. For immunofluorescence start at 20 μg/mL.
Antibody validated: Western Blot in human samples; Immunohistochemistry in mouse samples and Immunofluorescence in mouse samples. All other applications and species not yet tested. - POSITIVE CONTROL 1: Cat. No. 1211 - HepG2 Cell Lysate
- PREDICTED MOLECULAR WEIGHT: Predicted: 140 kDa
Observed: 150 kDa
Properties
- PURIFICATION: NPC1 Antibody is affinity chromatography purified via peptide column.
- CLONALITY: Polyclonal
- ISOTYPE: IgG
- PHYSICAL STATE: Liquid
- BUFFER: NPC1 Antibody is supplied in PBS containing 0.02% sodium azide.
- CONCENTRATION: 1 mg/mL
- STORAGE CONDITIONS: NPC1 antibody can be stored at 4°C for three months and -20°C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.
Additional Info
- NCBI OFFICIAL SYMBOL: NPC1
- ADDITIONAL NAMES: NPC1 Antibody: NPC, Niemann-Pick C1 protein
- Protein Accession Number: NP_000262
- PROTEIN GI NUMBER: 255652944
- NCBI GENE ID NUMBER: 4864
- USER NOTE: Optimal dilutions for each application to be determined by the researcher.
Background
- NPC1 Antibody: Mutations in the Niemann-Pick disease type C1 (NPC1) gene result in a fatal progressive neurodegenerative disorder characterized by an abnormal sequestration of lipids including cholesterol and glycosphingolipids. The NPC1 protein is 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. NPC1 transports low-density lipoproteins to late endosomal/lysosomal compartments where they are hydrolized and released as free cholesterol. NPC1, in addition to FTO, MC4R, and PTER has recently been shown to be a new risk loci for early-onset and morbid adult obesity in European populations. This anti-NPC1 antibody will not cross-react to NPC2, another gene whose defects also result in Niemann-Pick type C disease.
- 1: Karten B, Peake KB, and Vance JE. Mechanisms and consequences of impaired lipid trafficking in Niemann-Pick type C1-deficient mammalian cells. Biochim. Biophys. Acta2009; 1791:656-70.
- 2: Carstea ED, Polymeropoulos MH, Parker CC, et al. Linkage of Niemann-Pick disease type C to human chromosome 18. Proc. Natl. Acad. Sci. USA1993; 90:2002-4.
- 3: Carstea ED, Morris JA, Coleman KG, et al. Niemann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis. Science1977; 277:228-31.
- 4: Garver WS and Heidenreich RA. The Niemann-Pick C proteins and trafficking of cholesterol through the late endosomal/lysosomal system. Curr. Mol. Med.2002; 2:485-505.
Disclaimer
- FOR RESEARCH USE ONLY
For additional information, visit ProSci's Terms & Conditions Page. - Disclaimer: Optimal dilutions/concentrations should be determined by the end user. The information provided is a guideline for product use. This product is for research use only.
Shipping Info
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