Niemann-Pick C1 / Unconjugated /

Product Details
Supplier Boster Biological Technology
Catalog #: A00428-2 (View supplier product page)
Size 100 μg
Price $240.00
Antigen Niemann-Pick C1
Clone
Host Rabbit
Isotype IgG
Conjugate Unconjugated
Target Species Human, Mouse, Rat
Applications ELISA, WB
Description Rabbit IgG polyclonal antibody for Niemann Pick C1 detection. Tested with WB, Direct ELISA in Human;Mouse;Rat.
About Niemann-Pick C1 and Purified
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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Purified Excitation and Emission Spectra