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Home / Primary Antibodies

NPC1 Antibody / Niemann Pick C1 (RQ4395)

SKU : BHA17109215

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NSJ Bioreagents

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Mfr.No.	RQ4395
Clonality	Polyclonal
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.
Formulation	0.5mg/ml if reconstituted with 0.2ml sterile DI water
Host	Rabbit
Immunogen Region	A human partial recombinant protein corresponding to amino acids A1022-F1278 was used as the immunogen for the NPC1 antibody.
Isotype	IgG
Predicted Reactivity	Human, Mouse, Rat
Reactivity	Human, Mouse, Rat
Recombinant	No

Uniprot	O15118

Buffer	Lyophilized from 1X PBS with 2% Trehalose and 0.025% sodium azide
Format	Antigen affinity purified
Purification	Antigen affinity purified
Storage	After reconstitution, the NPC1 antibody can be stored for up to one month at 4°C. For long-term, aliquot and store at -20°C. Avoid repeated freezing and thawing.

Applications	WB, ELISA
Dilution	Weste blot: 0.5-1ug/ml,Direct ELISA: 0.1-0.5ug/ml (recombinant human protein) (BSA-free format available)

Product Manual	Data Sheet

No Description Available.

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SKU	Supplier No.	Size	Your price	Quantity
BHA17109215	RQ4395	100 ug	$419		Add to Cart

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