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Home >> Antibodies >> NPC1 Antibody / Niemann Pick C1

NPC1 Antibody / Niemann Pick C1 (RQ4395)

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  Catalog No Formulation Size Price (USD)  
Image RQ4395 0.5mg/ml if reconstituted with 0.2ml sterile DI water 100 ug 429
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Western blot testing of 1) rat pancreas and 2) mouse NIH 3T3 lysate with NPC1 antibody at 0.5ug/ml. Expected molecular weight: 140-190 kDa depending on glycosylation level.
Western blot testing of 1) rat pancreas and 2) mouse NIH 3T3 lysate with NPC1 antibody at 0.5ug/ml. Expected molecular weight: 140-190 kDa depending on glycosylation level.
Availability 1-3 business days
Species Reactivity Human, Mouse, Rat
Format Antigen affinity purified
Clonality Polyclonal (rabbit origin)
Isotype Rabbit IgG
Purity Antigen affinity purified
Buffer Lyophilized from 1X PBS with 2% Trehalose and 0.025% sodium azide
UniProt O15118
Applications Western blot : 0.5-1ug/ml
Direct ELISA : 0.1-0.5ug/ml (recombinant human protein) (BSA-free format available)
Limitations This NPC1 antibody is available for research use only.
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Description

Niemann-Pick disease, type C1 (NPC1) is a membrane protein that mediates intracellular cholesterol trafficking in mammals. In humans it is encoded by the NPC1 gene (chromosome location 18q11). 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.

Application Notes

Optimal dilution of the NPC1 antibody should be determined by the researcher.

Immunogen

A human partial recombinant protein corresponding to amino acids A1022-F1278 was used as the immunogen for the NPC1 antibody.

Storage

After reconstitution, the NPC1 antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.

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