PMID-sentid Pub_year Sent_text comp_official_name comp_offsetprotein_name organism prot_offset 26239048-3 2015 We aimed to know the sensitivity and specificity of these biomarkers for the diagnosis of NPC compared with other diseases that can potentially lead to oxysterol alterations. Oxysterols 152-161 NPC intracellular cholesterol transporter 1 Homo sapiens 90-93 10751394-10 2000 Finally, we showed that the lysosomal cholesterol pool in NP-C cells was substantially and preferentially reduced by incubating cells with the oxysterols, 25-hydroxycholesterol and 7-ketocholesterol; these findings suggest a new pharmacological approach to the treatment of NP-C disease. Oxysterols 143-153 NPC intracellular cholesterol transporter 1 Homo sapiens 58-62 26733147-7 2016 Interestingly, in NP-C alone, we observed that plasma oxysterols correlate negatively with patient"s age and positively with serum total bilirubin, suggesting the potential relationship between oxysterol levels and hepatic disease status. Oxysterols 54-63 NPC intracellular cholesterol transporter 1 Homo sapiens 18-22 12719428-6 2003 Moreover, we demonstrate that treatment with oxysterols reduces cholesterol in NPC mutants and is able to correct the NPC1I1061T phenotype, the most prevalent NPC1 disease genotype. Oxysterols 45-55 NPC intracellular cholesterol transporter 1 Homo sapiens 118-122 33315900-3 2020 We previously identified a series of oxysterol derivatives and phenanthridine-6-one derivatives as pharmacological chaperones, i.e., small molecules that can rescue folding-defective phenotypes of mutated NPC1, opening up an avenue to develop chaperone therapy for Niemann-Pick disease type C. Here, we present an improved image-based screen for NPC1 chaperones and we describe its application for drug-repurposing screening. Oxysterols 37-46 NPC intracellular cholesterol transporter 1 Homo sapiens 205-209 33315900-3 2020 We previously identified a series of oxysterol derivatives and phenanthridine-6-one derivatives as pharmacological chaperones, i.e., small molecules that can rescue folding-defective phenotypes of mutated NPC1, opening up an avenue to develop chaperone therapy for Niemann-Pick disease type C. Here, we present an improved image-based screen for NPC1 chaperones and we describe its application for drug-repurposing screening. Oxysterols 37-46 NPC intracellular cholesterol transporter 1 Homo sapiens 346-350 27147587-4 2016 The bile acids most elevated in the NPC subjects were identified as 3beta,5alpha,6beta-trihydroxycholanic acid and its glycine conjugate, which were shown to be metabolites of cholestane-3beta,5alpha,6beta-triol, an oxysterol elevated in NPC. Oxysterols 216-225 NPC intracellular cholesterol transporter 1 Homo sapiens 36-39 23521797-0 2013 Discovery of oxysterol-derived pharmacological chaperones for NPC1: implication for the existence of second sterol-binding site. Oxysterols 13-22 NPC intracellular cholesterol transporter 1 Homo sapiens 62-66 25095726-12 2015 CONCLUSION: Whilst acknowledging the limitations of this study, we conclude that screening ID children and adolescents with oxysterol tests compared to current practice for the diagnosis of NP-C is a dominant strategy with clinical and economic benefits. Oxysterols 124-133 NPC intracellular cholesterol transporter 1 Homo sapiens 190-194 24928400-5 2014 Here, we disclose detailed structure-activity relationships of oxysterol derivatives as pharmacological chaperones for NPC1(I1061T) mutant. Oxysterols 63-72 NPC intracellular cholesterol transporter 1 Homo sapiens 119-123 23521797-5 2013 These oxysterol derivatives bind to a domain of NPC1 that is different from the known N-terminal sterol-binding domain; i.e., there is an additional sterol-binding site on NPC1. Oxysterols 6-15 NPC intracellular cholesterol transporter 1 Homo sapiens 48-52 23521797-5 2013 These oxysterol derivatives bind to a domain of NPC1 that is different from the known N-terminal sterol-binding domain; i.e., there is an additional sterol-binding site on NPC1. Oxysterols 6-15 NPC intracellular cholesterol transporter 1 Homo sapiens 172-176 17989073-5 2008 Unexpectedly, we encountered NPC1 in a search for a membrane protein that binds 25-hydroxycholesterol (25-HC) and other oxysterols. Oxysterols 120-130 NPC intracellular cholesterol transporter 1 Homo sapiens 29-33 17989073-7 2008 We prepared recombinant human NPC1 and confirmed its ability to bind oxysterols, including those with a hydroxyl group on the 24, 25, or 27 positions. Oxysterols 69-79 NPC intracellular cholesterol transporter 1 Homo sapiens 30-34 17989073-13 2008 The availability of assays to measure NPC1 binding in vitro may further the understanding of ways in which oxysterols regulate intracellular lipid transport. Oxysterols 107-117 NPC intracellular cholesterol transporter 1 Homo sapiens 38-42