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NPC1, ID (NPC1, Niemann-Pick C1 protein) (FITC)

Cat no: 039083-FITC

NPC1, ID (NPC1, Niemann-Pick C1 protein) (FITC)

This gene encodes 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. It is predicted to have a cytoplasmic C-terminus, 13 transmembrane domains, and 3 large loops in the lumen of the endosome - the last loop being at the N-terminus. This protein transports low-density lipoproteins to late endosomal/lysosomal compartments where they are hydrolized and released as free cholesterol. Defects in this gene cause Niemann-Pick type C disease, a rare autosomal recessive neurodegenerative disorder characterized by over accumulation of cholesterol and glycosphingolipids in late endosomal/lysosomal compartments.\n\nApplications:\nSuitable for use in Western Blot, Immunohistochemistry, Immunofluorescence, ELISA\n\nRecommended Dilution:\nELISA: 1:1,000\nWestern Blot: 1:100-500\nImmunofluorescence: 1:10-50\nImmunohistochemistry: 1:10-50\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Do not freeze FITC conjugates. Light sensitive. Aliquots are stable for 6 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

039083-FITC

Size

200ul

Applications

ELISA, IF, IHC, WB

Hosts

Rabbit

Reactivities

Hum

Form

Supplied as a liquid in PBS, pH 7.2. Contains ~0.09% sodium azide. Conjugated to FITC.

P Type

Pab

Purity

Purified by Protein A affinity chromatography.

Isotype

IgG

References

Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010)\nFontaine-Bisson, B., et al. Diabetologia 53(10):2155-2162(2010)\nKagedal, K., et al. Biochim. Biophys. Acta 1801(8):831-838(2010)\nRodriguez-Rodriguez, E., et al. J. Alzheimers Dis. 21(2):619-625(2010)\nMa, W., et al. BMC Med. Genet. 11, 149 (2010) :

Additional Info

Human

Alternative Names

NPC1, Niemann-Pick C1 protein

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