Technical Data

146447
Clone Type
Polyclonal
Host
Rabbit
Source
Human
Isotype
IgG
Grade
Affinity Purified
Applications
E IF IHC WB
Crossreactivity
Hu
Shipping Temp
Blue Ice
Storage Temp
-20°C
Rabbit Anti-NPC1 (Niemann-Pick C1 Protein)

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.

Applications
Suitable for use in ELISA, Western Blot, Immunofluorescence and Immunohistochemistry. Other applications not tested.
Recommended Dilution
Western Blot: 1ug/ml Immunohistochemistry (Formalin fixed paraffin embedded): 2.5ug/ml Immunofluorescence: 20ug/ml Optimal dilutions to be determined by the researcher.
Positive Control
Human Kidney Tissue Lysate
Storage and Stability
May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Immunogen
Synthetic peptide corresponding to 16aa from near the C-terminus of human NPC1.
Form
Supplied as a liquid in PBS, 0.02% sodium azide.
Purity
Purified by immunoaffinity chromatography.
Specificity
Recognizes human NPC1.

Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.

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.
USBio References
No references available
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