Title : Complex lipid trafficking in Niemann-Pick disease type C.

Pub. Date : 2015 Jan

PMID : 25425283






1 Functional Relationships(s)
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1 It summarizes the current knowledge on the NPC1 and NPC2 proteins and their function in transport of cholesterol within the late endosomal-lysosomal compartment, with emphasis on differences between systemic organs and the brain; it also discusses regulation by membrane lipids of the NPC2-mediated cholesterol trafficking, interplay between cholesterol and sphingomyelin, the metabolic origin of glycosphingolipids stored in brain, and the putative role of free sphingoid bases in pathogenesis. Sphingosine NPC intracellular cholesterol transporter 1 Homo sapiens