Niemann-Pick Disease, Type C1 (NPC1, Niemann-Pick C1, Niemann-Pick C1 Protein Precursor, NPC)

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.\nNPC 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.\n\nPositive Control: Human fibroblast cell lysate\n\nApplications: \nSuitable for use in Immunofluorescence, Western Blot, Immunoprecipitation and Immunohistochemistry. Other applications not tested.\n\nRecommended Dilution:\nWestern Blot: Detecting heterogeneously glycosylated NPC1 protein with prominent bands at 170 and 220kD.\nOptimal dilutions to be determined by the researcher.\n\nStorage and Stability:\n\nMay be stored at 4 degrees C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot and store at -20 degrees C. Aliquots are stable for at least 12 months at -20 degrees C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

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SPECIFICATIONS

Catalog Number

N2566-31A

Size

100ul

Applications

IF, IHC, IP, WB

Hosts

Rabbit

Reactivities

Hum, Mouse

Form

Supplied as a liquid in PBS, pH 7.2.

P Type

Pab

Purity

Purified by immunoaffinity chromatography.

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]\n2. 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]

Additional Info

This antibody is specific for NPC1 protein. Species Crossreactivity: This antibody reacts with human, mouse, and hamster protein.