Rabbit Polyclonal Antibody to NPC1

包装: 1G|100G|500G|1KG|100KG

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别名:Niemann-Pick C1 protein, NPC1
应用:WB,IHC,ICC
反应种属:Human, Mouse
规格：50μl/100μl

Description
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.

Description

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.

Specification
Aliases	Niemann-Pick C1 protein, NPC1
Entrez GeneID	4864
Swissprot	O15118
WB Predicted band size	142.2kDa
Host/Isotype	Rabbit IgG
Storage	Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species Reactivity	Human, Mouse
Immunogen	This NPC1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 591-620 amino acids from the Central region of human NPC1.
Formulation	Purified polyclonal antibody supplied in PBS with 0.05% sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Application
WB	1/1000-1/2000
IHC	1/100-1/500
ICC	1/10-1/50

	Anti-NPC1 Antibody (Center) at 1:2000 dilution + Hela whole cell lysate Lysates/proteins at 20 µg per lane. Secondary Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution. Predicted band size : 142 kDa Blocking/Dilution buffer: 5% NFDM/TBST.
	Anti-NPC1 Antibody (Center) at 1:1000 dilution + NCI-H460 whole cell lysate Lysates/proteins at 20 µg per lane. Secondary Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution. Predicted band size : 142 kDa Blocking/Dilution buffer: 5% NFDM/TBST.
	NPC1 Antibody (Center) (Cat. #P34840) western blot analysis in NCI-H460 cell line lysates (35ug/lane).This demonstrates the NPC1 antibody detected the NPC1 protein (arrow).
	Confocal immunofluorescent analysis of NPC1 Antibody (Center) (Cat#P34840) with 293 cell followed by Alexa Fluor 488-conjugated goat anti-rabbit lgG (green). DAPI was used to stain the cell nuclear (blue).
	NPC1 Antibody (Center) (Cat. #P34840)immunohistochemistry analysis in formalin fixed and paraffin embedded human kidney tissue followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of NPC1 Antibody (Center) for immunohistochemistry. Clinical relevance has not been evaluated.