Catalog# :5185
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.
Additional Names : NPC1, Niemann-Pick disease type C1, NPC
Description
Left: Western blot analysis of NPC1 in human kidney tissue lysate with NPC1 antibody at 1 µg/ml.
Below:Immunohistochemistry of NPC1 in mouse kidney tissue with NPC1 antibody at 2.5 μg/ml.
Other Product Images
Description
Left: Western blot analysis of NPC1 in human kidney tissue lysate with NPC1 antibody at 1 µg/ml.
Below:Immunohistochemistry of NPC1 in mouse kidney tissue with NPC1 antibody at 2.5 μg/ml.
Other Product Images
Source :NPC1 antibody was raised against a 16 amino acid peptide from near the carboxy terminus of human NPC1.
Purification : Affinity chromatography purified via peptide column
Clonality and Clone : This is a polyclonal antibody.
Host : NPC1 antibody was raised in rabbit.
Please use anti-rabbit secondary antibodies.
Application : NPC1 antibody can be used for detection of NPC1 by Western blot at 1 - 2 µg/ml.
Tested Application(s) : E, WB, IHC
Buffer : Antibody is supplied in PBS containing 0.02% sodium azide.
Blocking Peptide :Cat.No. 5185P - NPC1 Peptide
Long-Term Storage : NPC1 antibody can be stored at 4ºC, stable for 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.
Positive Control :
1. Cat. No. 1305 - Human Kidney Tissue Lysate
Species Reactivity : H
GI Number : 255652944
Accession Number : NP_000262
Short Description : Niemann-Pick disease type C1
References
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. Acta 2009; 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. USA 1993; 90:2002-4.
3. Carstea ED, Morris JA, Coleman KG, et al. Niemann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis. Science 1977; 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.
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