PMID-sentid Pub_year Sent_text comp_official_name comp_offsetprotein_name organism prot_offset 8204660-3 1994 In liver and spleen from NPC patients, a 6- to 24-fold elevation of sphingosine and sphinganine already prominent at the fetal stage of the disease was observed, while no clear increase could be evidenced in brain tissue. Sphingosine 68-79 NPC intracellular cholesterol transporter 1 Homo sapiens 25-28 25946402-6 2016 Niemann-Pick disease type C is a rare, devastating, inherited lysosomal storage disease with a unique cellular phenotype characterized by lysosomal accumulation of sphingosine, various glycosphingolipids and cholesterol and a reduction in lysosomal calcium. Sphingosine 164-175 NPC intracellular cholesterol transporter 1 Homo sapiens 0-27 34445564-4 2021 The first trait of NPC is the accumulation of unesterified cholesterol and other lipids, like sphingosine and glycosphingolipids, in the late endosomal and lysosomal compartments, which causes the blockade of autophagic flux and the impairment of mitochondrial functions. Sphingosine 94-105 NPC intracellular cholesterol transporter 1 Homo sapiens 19-22 33044062-7 2020 Using our probe, we successfully detected sphingosine accumulation in cells from patients with Niemann-Pick type C1 (NPC1), a lipid transport disorder in which increased sphingosine mediates disease progression. Sphingosine 42-53 NPC intracellular cholesterol transporter 1 Homo sapiens 95-115 33044062-7 2020 Using our probe, we successfully detected sphingosine accumulation in cells from patients with Niemann-Pick type C1 (NPC1), a lipid transport disorder in which increased sphingosine mediates disease progression. Sphingosine 42-53 NPC intracellular cholesterol transporter 1 Homo sapiens 117-121 33044062-7 2020 Using our probe, we successfully detected sphingosine accumulation in cells from patients with Niemann-Pick type C1 (NPC1), a lipid transport disorder in which increased sphingosine mediates disease progression. Sphingosine 170-181 NPC intracellular cholesterol transporter 1 Homo sapiens 95-115 33044062-7 2020 Using our probe, we successfully detected sphingosine accumulation in cells from patients with Niemann-Pick type C1 (NPC1), a lipid transport disorder in which increased sphingosine mediates disease progression. Sphingosine 170-181 NPC intracellular cholesterol transporter 1 Homo sapiens 117-121 21344635-1 2011 Niemann-Pick disease type C (NPC) is a rare autosomal recessive lysosomal storage disorder characterized by defective intracellular lipid trafficking, with secondary accumulation of free cholesterol, sphingosine, and glycosphingolipids. Sphingosine 200-211 NPC intracellular cholesterol transporter 1 Homo sapiens 29-32 25425283-7 2015 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 463-472 NPC intracellular cholesterol transporter 1 Homo sapiens 43-47 22607065-7 2012 Additional evidence against NPC1 playing a significant role in LE/LY sphingosine export was obtained in experiments using the [(3)H]-sphingolipids or a fluorescent sphingosine derivative in NPC1 knock-out cells. Sphingosine 69-80 NPC intracellular cholesterol transporter 1 Homo sapiens 28-32 22607065-9 2012 This likely contributes to the increased sphingosine content of NPC1 cells. Sphingosine 41-52 NPC intracellular cholesterol transporter 1 Homo sapiens 64-68 21344635-1 2011 Niemann-Pick disease type C (NPC) is a rare autosomal recessive lysosomal storage disorder characterized by defective intracellular lipid trafficking, with secondary accumulation of free cholesterol, sphingosine, and glycosphingolipids. Sphingosine 200-211 NPC intracellular cholesterol transporter 1 Homo sapiens 0-27 23881911-7 2013 Plasma MCs and ceramides were elevated, whereas sphingoid bases were reduced in NPC1 subjects. Sphingosine 48-57 NPC intracellular cholesterol transporter 1 Homo sapiens 80-84