Rabbit anti-Human Niemann Pick C1 Monoclonal Antibody-Monoclonal Antibody-Biotyscience

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Rabbit anti-Human Niemann Pick C1 Monoclonal Antibody

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Cat No	BMA-2314
Conjugate
Type	重组兔多抗
Source	Rabbit
Size	50 uL
Application	WB; IHC
Format	Liquid
Concentration	Please refer to the vial lable for the specific concentration.
Buffer	Supplied in PBS with 0.02% sodium azide,50% glycerol,pH7.3.
Species	Human
Storage	Store at -20℃. Avoid freeze / thaw cycles.
Synonyms	NPC1;NPC;Niemann-Pick C1 protein
Purification	Affinity purification
MolecularWeight	180KDa
Description
Background	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.

We can offer labeled proteins/antibodies using a broad range of intensely fluorescent dyes and labels including FITC, Biotin, Alexa Fluor, Rhodamine B, Cy, Co-Au.

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Rabbit anti-Human Niemann Pick C1 Monoclonal Antibody