Niemann-Pick C1 / HRP /

Product Details
Supplier Biorbyt
Catalog #: orb415306-50ug (View supplier product page)
Size 50 μg
Price $343.40
Antigen Niemann-Pick C1
Clone
Host Rabbit
Isotype IgG
Conjugate HRP
Target Species Human
Applications ELISA
Description Rabbit polyclonal antibody against NPC1 conjugated to HRP
About Niemann-Pick C1 and HRP
Niemann-Pick C1 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.[provided by RefSeq, Aug 2009]
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HRP Excitation and Emission Spectra