PMID-sentid Pub_year Sent_text comp_official_name comp_offsetprotein_name organism prot_offset 28860124-7 2017 Our study shows that treatment of NPC1I1061T fibroblasts with Vorinostat not only enhances pathways promoting the folding, stabilization and trafficking of NPC1 (I1061T) mutant to the LE/Ly, but alters the expression of lysosomal proteins, specifically the lysosomal acid lipase (LIPA) involved in the LIPA->NPC2->NPC1 based flow of cholesterol from the LE/Ly lumen to the LE/Ly membrane. Vorinostat 62-72 NPC intracellular cholesterol transporter 1 Homo sapiens 34-38 28860124-8 2017 We posit that the Vorinostat may modulate numerous pathways that operate in an integrated fashion through epigenetic and post-translational modifications reflecting acetylation/deacetylation balance to help manage the defective NPC1 fold, the function of the LE/Ly system and/or additional cholesterol metabolism/distribution pathways, that could globally contribute to improved mitigation of NPC1 disease in the clinic based on as yet uncharacterized principles of cellular metabolism dictating cholesterol homeostasis. Vorinostat 18-28 NPC intracellular cholesterol transporter 1 Homo sapiens 228-232 28860124-7 2017 Our study shows that treatment of NPC1I1061T fibroblasts with Vorinostat not only enhances pathways promoting the folding, stabilization and trafficking of NPC1 (I1061T) mutant to the LE/Ly, but alters the expression of lysosomal proteins, specifically the lysosomal acid lipase (LIPA) involved in the LIPA->NPC2->NPC1 based flow of cholesterol from the LE/Ly lumen to the LE/Ly membrane. Vorinostat 62-72 NPC intracellular cholesterol transporter 1 Homo sapiens 156-160 28193631-6 2017 Here, we show that several other human NPC1 mutant fibroblast cell lines can also be corrected by vorinostat or panobinostat and that treatment with vorinostat extends the lifetime of the NPC1I1061T protein. Vorinostat 98-108 NPC intracellular cholesterol transporter 1 Homo sapiens 39-43 28193631-5 2017 Treatment of patient-derived NPC1I1061T fibroblasts with histone deacetylase inhibitors (HDACis) vorinostat or panobinostat increases expression of the mutant NPC1 protein and leads to correction of the cholesterol storage. Vorinostat 97-107 NPC intracellular cholesterol transporter 1 Homo sapiens 29-33 28193631-6 2017 Here, we show that several other human NPC1 mutant fibroblast cell lines can also be corrected by vorinostat or panobinostat and that treatment with vorinostat extends the lifetime of the NPC1I1061T protein. Vorinostat 149-159 NPC intracellular cholesterol transporter 1 Homo sapiens 39-43 28031458-2 2017 Vorinostat, an FDA-approved inhibitor of histone deacetylases, ameliorates lysosomal lipid accumulation in cultured NP-C patient fibroblasts. Vorinostat 0-10 NPC intracellular cholesterol transporter 1 Homo sapiens 116-120