{"id":"https://openalex.org/W2063467367","doi":"https://doi.org/10.1371/journal.pone.0028777","title":"Alteration of Gene Expression Profile in Niemann-Pick Type C Mice Correlates with Tissue Damage and Oxidative Stress","display_name":"Alteration of Gene Expression Profile in Niemann-Pick Type C Mice Correlates with Tissue Damage and Oxidative Stress","publication_year":2011,"publication_date":"2011-12-22","ids":{"openalex":"https://openalex.org/W2063467367","doi":"https://doi.org/10.1371/journal.pone.0028777","mag":"2063467367","pmid":"https://pubmed.ncbi.nlm.nih.gov/22216111","pmcid":"https://www.ncbi.nlm.nih.gov/pmc/articles/3245218"},"language":"en","primary_location":{"id":"doi:10.1371/journal.pone.0028777","doi":"https://doi.org/10.1371/journal.pone.0028777","is_oa":true,"landing_page_url":"https://doi.org/10.1371/journal.pone.0028777","pdf_url":"https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0028777&type=printable","source":{"id":"https://openalex.org/S202381698","display_name":"PLoS ONE","issn_l":"1932-6203","issn":["1932-6203"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["anvur-class-a","anvur-scientific","cwts-core","doaj","dongbi-c","doyens","erih-plus","jufo-1","ki-jl-1","medline","norway-1","poland-100","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310315706","host_organization_name":"Public Library of Science","host_organization_lineage":["https://openalex.org/P4310315706"],"host_organization_lineage_names":["Public Library of Science"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"PLoS ONE","raw_type":"journal-article"},"volume":null,"type":"article","is_traditional":true,"indexed_in":["crossref","doaj","pubmed"],"open_access":{"is_oa":true,"oa_status":"gold","oa_url":"https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0028777&type=printable","any_repository_has_fulltext":true},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5048417671","display_name":"Mary Carmen V\u00e1zquez","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I162148367","display_name":"Pontificia Universidad Cat\u00f3lica de Chile","ror":"https://ror.org/04teye511","country_code":"CL","type":"education","lineage":["https://openalex.org/I162148367"]}],"countries":["CL"],"is_corresponding":false,"raw_author_name":"Mary C. V\u00e1zquez","raw_affiliation_strings":["Departamento de Gastroenterolog\u00eda, Facultad de Medicina, Pontificia Universidad Cat\u00f3lica de Chile, Santiago, Chile"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Departamento de Gastroenterolog\u00eda, Facultad de Medicina, Pontificia Universidad Cat\u00f3lica de Chile, Santiago, Chile","institution_ids":["https://openalex.org/I162148367"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5032724757","display_name":"Tal\u00eda del Pozo","orcid":"https://orcid.org/0000-0002-9169-2529","observed_orcids":["https://orcid.org/0000-0002-9169-2529"]},"institutions":[{"id":"https://openalex.org/I69737025","display_name":"University of Chile","ror":"https://ror.org/047gc3g35","country_code":"CL","type":"education","lineage":["https://openalex.org/I69737025"]}],"countries":["CL"],"is_corresponding":false,"raw_author_name":"Tal\u00eda del Pozo","raw_affiliation_strings":["FONDAP-Center of Genome Regulation (CGR), Santiago, Chile","Laboratorio de Bioinform\u00e1tica y Expresi\u00f3n G\u00e9nica, INTA, Universidad de Chile, Santiago, Chile"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"FONDAP-Center of Genome Regulation (CGR), Santiago, Chile","institution_ids":[]},{"raw_affiliation_string":"Laboratorio de Bioinform\u00e1tica y Expresi\u00f3n G\u00e9nica, INTA, Universidad de Chile, Santiago, Chile","institution_ids":["https://openalex.org/I69737025"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5026998949","display_name":"Ferm\u00edn Robledo","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I162148367","display_name":"Pontificia Universidad Cat\u00f3lica de Chile","ror":"https://ror.org/04teye511","country_code":"CL","type":"education","lineage":["https://openalex.org/I162148367"]}],"countries":["CL"],"is_corresponding":false,"raw_author_name":"Ferm\u00edn A. Robledo","raw_affiliation_strings":["Departamento de Gastroenterolog\u00eda, Facultad de Medicina, Pontificia Universidad Cat\u00f3lica de Chile, Santiago, Chile"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Departamento de Gastroenterolog\u00eda, Facultad de Medicina, Pontificia Universidad Cat\u00f3lica de Chile, Santiago, Chile","institution_ids":["https://openalex.org/I162148367"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5029561674","display_name":"Gonzalo Carrasco-Avi\u00f1o","orcid":"https://orcid.org/0000-0003-2415-5413","observed_orcids":["https://orcid.org/0000-0003-2415-5413"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Gonzalo Carrasco","raw_affiliation_strings":["Fundaci\u00f3n Hospital Parroquial de San Bernardo, Santiago, Chile"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Fundaci\u00f3n Hospital Parroquial de San Bernardo, Santiago, Chile","institution_ids":[]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5090354366","display_name":"Leonardo Pav\u00e9z","orcid":"https://orcid.org/0000-0003-1093-8134","observed_orcids":["https://orcid.org/0000-0003-1093-8134"]},"institutions":[{"id":"https://openalex.org/I69737025","display_name":"University of Chile","ror":"https://ror.org/047gc3g35","country_code":"CL","type":"education","lineage":["https://openalex.org/I69737025"]}],"countries":["CL"],"is_corresponding":false,"raw_author_name":"Leonardo Pavez","raw_affiliation_strings":["Laboratorio de Bioinform\u00e1tica y Expresi\u00f3n G\u00e9nica, INTA, Universidad de Chile, Santiago, Chile"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Laboratorio de Bioinform\u00e1tica y Expresi\u00f3n G\u00e9nica, INTA, Universidad de Chile, Santiago, Chile","institution_ids":["https://openalex.org/I69737025"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5056619917","display_name":"Felipe Olivares","orcid":"https://orcid.org/0000-0002-4798-8680","observed_orcids":["https://orcid.org/0000-0002-4798-8680"]},"institutions":[{"id":"https://openalex.org/I69737025","display_name":"University of Chile","ror":"https://ror.org/047gc3g35","country_code":"CL","type":"education","lineage":["https://openalex.org/I69737025"]}],"countries":["CL"],"is_corresponding":false,"raw_author_name":"Felipe Olivares","raw_affiliation_strings":["Laboratorio de Bioinform\u00e1tica y Expresi\u00f3n G\u00e9nica, INTA, Universidad de Chile, Santiago, Chile"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Laboratorio de Bioinform\u00e1tica y Expresi\u00f3n G\u00e9nica, INTA, Universidad de Chile, Santiago, Chile","institution_ids":["https://openalex.org/I69737025"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5011502661","display_name":"Maur\u00edcio Gonz\u00e1lez","orcid":"https://orcid.org/0000-0002-1592-9758","observed_orcids":["https://orcid.org/0000-0002-1592-9758"]},"institutions":[{"id":"https://openalex.org/I4387155550","display_name":"Center for Mathematical Modeling","ror":"https://ror.org/00wz2vk41","country_code":"CL","type":"facility","lineage":["https://openalex.org/I1294671590","https://openalex.org/I4387155550","https://openalex.org/I69737025"]},{"id":"https://openalex.org/I69737025","display_name":"University of Chile","ror":"https://ror.org/047gc3g35","country_code":"CL","type":"education","lineage":["https://openalex.org/I69737025"]}],"countries":["CL"],"is_corresponding":false,"raw_author_name":"Mauricio Gonz\u00e1lez","raw_affiliation_strings":["FONDAP-Center of Genome Regulation (CGR), Santiago, Chile","Laboratorio de Bioinform\u00e1tica y Expresi\u00f3n G\u00e9nica, INTA, Universidad de Chile, Santiago, Chile","Laboratorio de Bioinform\u00e1tica y Matem\u00e1ticas del Genoma, Centro de Modelamiento Matem\u00e1tico (CMM), Facultad de Ciencias F\u00edsicas y Matem\u00e1ticas, Universidad de Chile, Santiago, Chile"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"FONDAP-Center of Genome Regulation (CGR), Santiago, Chile","institution_ids":[]},{"raw_affiliation_string":"Laboratorio de Bioinform\u00e1tica y Expresi\u00f3n G\u00e9nica, INTA, Universidad de Chile, Santiago, Chile","institution_ids":["https://openalex.org/I69737025"]},{"raw_affiliation_string":"Laboratorio de Bioinform\u00e1tica y Matem\u00e1ticas del Genoma, Centro de Modelamiento Matem\u00e1tico (CMM), Facultad de Ciencias F\u00edsicas y Matem\u00e1ticas, Universidad de Chile, Santiago, Chile","institution_ids":["https://openalex.org/I4387155550","https://openalex.org/I69737025"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5061046567","display_name":"Silvana Zanlungo","orcid":"https://orcid.org/0000-0001-8383-9829","observed_orcids":["https://orcid.org/0000-0001-8383-9829"]},"institutions":[{"id":"https://openalex.org/I162148367","display_name":"Pontificia Universidad Cat\u00f3lica de Chile","ror":"https://ror.org/04teye511","country_code":"CL","type":"education","lineage":["https://openalex.org/I162148367"]}],"countries":["CL"],"is_corresponding":true,"raw_author_name":"Silvana Zanlungo","raw_affiliation_strings":["Departamento de Gastroenterolog\u00eda, Facultad de Medicina, Pontificia Universidad Cat\u00f3lica de Chile, Santiago, Chile","FONDAP-Center of Genome Regulation (CGR), Santiago, Chile"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Departamento de Gastroenterolog\u00eda, Facultad de Medicina, Pontificia Universidad Cat\u00f3lica de Chile, Santiago, Chile","institution_ids":["https://openalex.org/I162148367"]},{"raw_affiliation_string":"FONDAP-Center of Genome Regulation (CGR), Santiago, Chile","institution_ids":[]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":3,"corresponding_author_ids":["https://openalex.org/A5061046567"],"corresponding_institution_ids":["https://openalex.org/I162148367"],"apc_list":{"value":2382,"currency":"USD","value_usd":2382},"apc_paid":{"value":2382,"currency":"USD","value_usd":2382},"fwci":4.0701,"has_fulltext":true,"cited_by_count":63,"citation_normalized_percentile":{"value":0.93929712,"is_in_top_1_percent":false,"is_in_top_10_percent":true},"cited_by_percentile_year":{"min":88,"max":99},"biblio":{"volume":"6","issue":"12","first_page":"e28777","last_page":"e28777"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10945","display_name":"Lysosomal Storage Disorders Research","score":0.9951000213623047,"subfield":{"id":"https://openalex.org/subfields/2716","display_name":"Genetics (clinical)"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},"topics":[{"id":"https://openalex.org/T10945","display_name":"Lysosomal Storage Disorders Research","score":0.9951000213623047,"subfield":{"id":"https://openalex.org/subfields/2716","display_name":"Genetics (clinical)"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T12604","display_name":"Glycogen Storage Diseases and Myoclonus","score":0.0019000000320374966,"subfield":{"id":"https://openalex.org/subfields/1303","display_name":"Biochemistry"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T11027","display_name":"Metabolism and Genetic Disorders","score":0.0008999999845400453,"subfield":{"id":"https://openalex.org/subfields/1308","display_name":"Clinical Biochemistry"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/niemann-pick-disease-type-c","display_name":"Niemann-Pick disease type C","score":0.9850000143051147},{"id":"https://openalex.org/keywords/oxidative-stress","display_name":"oxidative stress","score":0.7680000066757202},{"id":"https://openalex.org/keywords/liver-fibrosis","display_name":"liver fibrosis","score":0.9330000281333923},{"id":"https://openalex.org/keywords/liver-inflammation","display_name":"liver inflammation","score":0.6769999861717224},{"id":"https://openalex.org/keywords/gene-expression-profiling","display_name":"gene expression profiling","score":0.621999979019165},{"id":"https://openalex.org/keywords/copper-metabolism","display_name":"copper metabolism","score":0.6389999985694885},{"id":"https://openalex.org/keywords/glutathione","display_name":"glutathione","score":0.8690000176429749},{"id":"https://openalex.org/keywords/protein-carbonylation","display_name":"protein carbonylation","score":0.9409999847412109},{"id":"https://openalex.org/keywords/unesterified-cholesterol-accumulation","display_name":"unesterified cholesterol accumulation","score":0.7570000290870667},{"id":"https://openalex.org/keywords/cerebellum","display_name":"cerebellum","score":0.8769999742507935},{"id":"https://openalex.org/keywords/lysosomal-lipid-storage-disorder","display_name":"lysosomal lipid storage disorder","score":0.6940000057220459}],"concepts":[{"id":"https://openalex.org/C2776151105","wikidata":"https://www.wikidata.org/wiki/Q898814","display_name":"Oxidative stress","level":2,"score":0.7441312074661255},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.5940424203872681},{"id":"https://openalex.org/C2778552423","wikidata":"https://www.wikidata.org/wiki/Q14914279","display_name":"NPC1","level":4,"score":0.5738463401794434},{"id":"https://openalex.org/C2776914184","wikidata":"https://www.wikidata.org/wiki/Q101991","display_name":"Inflammation","level":2,"score":0.5146997570991516},{"id":"https://openalex.org/C2780559512","wikidata":"https://www.wikidata.org/wiki/Q605709","display_name":"Fibrosis","level":2,"score":0.5003201961517334},{"id":"https://openalex.org/C2779652256","wikidata":"https://www.wikidata.org/wiki/Q130983","display_name":"Cerebellum","level":2,"score":0.4865797758102417},{"id":"https://openalex.org/C2779489717","wikidata":"https://www.wikidata.org/wiki/Q1419931","display_name":"Niemann\u2013Pick disease","level":3,"score":0.4691537618637085},{"id":"https://openalex.org/C2780635549","wikidata":"https://www.wikidata.org/wiki/Q2067267","display_name":"Niemann\u2013Pick disease, type C","level":3,"score":0.45836955308914185},{"id":"https://openalex.org/C134018914","wikidata":"https://www.wikidata.org/wiki/Q162606","display_name":"Endocrinology","level":1,"score":0.4173111319541931},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.367827832698822},{"id":"https://openalex.org/C142724271","wikidata":"https://www.wikidata.org/wiki/Q7208","display_name":"Pathology","level":1,"score":0.3284575045108795},{"id":"https://openalex.org/C2778163477","wikidata":"https://www.wikidata.org/wiki/Q43656","display_name":"Cholesterol","level":2,"score":0.2108856439590454},{"id":"https://openalex.org/C55493867","wikidata":"https://www.wikidata.org/wiki/Q7094","display_name":"Biochemistry","level":1,"score":0.17326200008392334},{"id":"https://openalex.org/C203014093","wikidata":"https://www.wikidata.org/wiki/Q101929","display_name":"Immunology","level":1,"score":0.16520896553993225},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.14668318629264832},{"id":"https://openalex.org/C102747710","wikidata":"https://www.wikidata.org/wiki/Q824237","display_name":"Endosome","level":3,"score":0.12554556131362915},{"id":"https://openalex.org/C79879829","wikidata":"https://www.wikidata.org/wiki/Q5571762","display_name":"Intracellular","level":2,"score":0.07880830764770508}],"mesh":[{"descriptor_ui":"D000077296","descriptor_name":"Niemann-Pick C1 Protein","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000818","descriptor_name":"Animals","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D004195","descriptor_name":"Disease Models, Animal","qualifier_ui":null,"qualifier_name":null,"is_major_topic":true},{"descriptor_ui":"D008807","descriptor_name":"Mice, Inbred BALB C","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D011506","descriptor_name":"Proteins","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D012333","descriptor_name":"RNA, Messenger","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D018345","descriptor_name":"Mice, Knockout","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D018384","descriptor_name":"Oxidative Stress","qualifier_ui":null,"qualifier_name":null,"is_major_topic":true},{"descriptor_ui":"D020869","descriptor_name":"Gene Expression Profiling","qualifier_ui":null,"qualifier_name":null,"is_major_topic":true},{"descriptor_ui":"D047908","descriptor_name":"Intracellular Signaling Peptides and Proteins","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D051379","descriptor_name":"Mice","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D052556","descriptor_name":"Niemann-Pick Disease, Type C","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":true},{"descriptor_ui":"D060888","descriptor_name":"Real-Time Polymerase Chain Reaction","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false}],"locations_count":18,"locations":[{"id":"doi:10.1371/journal.pone.0028777","doi":"https://doi.org/10.1371/journal.pone.0028777","is_oa":true,"landing_page_url":"https://doi.org/10.1371/journal.pone.0028777","pdf_url":"https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0028777&type=printable","source":{"id":"https://openalex.org/S202381698","display_name":"PLoS ONE","issn_l":"1932-6203","issn":["1932-6203"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["anvur-class-a","anvur-scientific","cwts-core","doaj","dongbi-c","doyens","erih-plus","jufo-1","ki-jl-1","medline","norway-1","poland-100","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310315706","host_organization_name":"Public Library of Science","host_organization_lineage":["https://openalex.org/P4310315706"],"host_organization_lineage_names":["Public Library of Science"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"PLoS ONE","raw_type":"journal-article"},{"id":"pmh:oai:europepmc.org:2258532","doi":null,"is_oa":true,"landing_page_url":"https://www.ncbi.nlm.nih.gov/pmc/articles/3245218","pdf_url":null,"source":{"id":"https://openalex.org/S4306400806","display_name":"Europe PMC (PubMed Central)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1303153112","host_organization_name":"European Bioinformatics Institute","host_organization_lineage":["https://openalex.org/I1303153112"],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":null,"raw_type":"Text"},{"id":"pmid:22216111","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/22216111","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"PloS one","raw_type":"Journal Article"},{"id":"pmh:oai:pubmedcentral.nih.gov:3245218","doi":null,"is_oa":true,"landing_page_url":"https://pmc.ncbi.nlm.nih.gov/articles/PMC3245218/","pdf_url":null,"source":{"id":"https://openalex.org/S2764455111","display_name":"PubMed Central","issn_l":null,"issn":null,"is_oa":true,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"PLoS One","raw_type":"Text"},{"id":"pmh:oai:CiteSeerX.psu:10.1.1.814.7919","doi":null,"is_oa":false,"landing_page_url":"http://citeseerx.ist.psu.edu/viewdoc/summary?doi=10.1.1.814.7919","pdf_url":null,"source":null,"license":null,"license_id":null,"version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"ftp://ftp.ncbi.nlm.nih.gov/pub/pmc/32/a1/pone.0028777.PMC3245218.pdf","raw_type":"text"},{"id":"pmh:oai:americanae.aecid.es:3269306","doi":null,"is_oa":true,"landing_page_url":"http://americanae.aecid.es/americanae/es/registros/registro.do?tipoRegistro=MTD&idBib=3269306","pdf_url":null,"source":{"id":"https://openalex.org/S4306400786","display_name":"Americanae (AECID Library)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"repository"},"license":"other-oa","license_id":"https://openalex.org/licenses/other-oa","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"PLOS ONE","raw_type":"info:eu-repo/semantics/article"},{"id":"pmh:oai:americanae.aecid.es:3269307","doi":null,"is_oa":true,"landing_page_url":"http://americanae.aecid.es/americanae/es/registros/registro.do?tipoRegistro=MTD&idBib=3269307","pdf_url":null,"source":{"id":"https://openalex.org/S4306400786","display_name":"Americanae (AECID Library)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"repository"},"license":"other-oa","license_id":"https://openalex.org/licenses/other-oa","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"PLOS ONE","raw_type":"info:eu-repo/semantics/article"},{"id":"pmh:oai:americanae.aecid.es:3269308","doi":null,"is_oa":true,"landing_page_url":"http://americanae.aecid.es/americanae/es/registros/registro.do?tipoRegistro=MTD&idBib=3269308","pdf_url":null,"source":{"id":"https://openalex.org/S4306400786","display_name":"Americanae (AECID Library)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"repository"},"license":"other-oa","license_id":"https://openalex.org/licenses/other-oa","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"PLOS ONE","raw_type":"info:eu-repo/semantics/article"},{"id":"pmh:oai:americanae.aecid.es:3451887","doi":null,"is_oa":true,"landing_page_url":"http://americanae.aecid.es/americanae/es/registros/registro.do?tipoRegistro=MTD&idBib=3451887","pdf_url":null,"source":{"id":"https://openalex.org/S4306400786","display_name":"Americanae (AECID Library)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by-nc-nd","license_id":"https://openalex.org/licenses/cc-by-nc-nd","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"Plos One","raw_type":"info:eu-repo/semantics/article"},{"id":"pmh:oai:americanae.aecid.es:3453354","doi":null,"is_oa":true,"landing_page_url":"http://americanae.aecid.es/americanae/es/registros/registro.do?tipoRegistro=MTD&idBib=3453354","pdf_url":null,"source":{"id":"https://openalex.org/S4306400786","display_name":"Americanae (AECID Library)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by-nc-nd","license_id":"https://openalex.org/licenses/cc-by-nc-nd","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"Plos One","raw_type":"info:eu-repo/semantics/article"},{"id":"pmh:oai:doaj.org/article:d14149d98b82404c9e28803aa98904b1","doi":null,"is_oa":true,"landing_page_url":"https://doaj.org/article/d14149d98b82404c9e28803aa98904b1","pdf_url":null,"source":{"id":"https://openalex.org/S4306401280","display_name":"DOAJ (DOAJ: Directory of Open Access Journals)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by-sa","license_id":"https://openalex.org/licenses/cc-by-sa","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"PLoS ONE, Vol 6, Iss 12, p e28777 (2011)","raw_type":"article"},{"id":"pmh:oai:figshare.com:article/130216","doi":null,"is_oa":true,"landing_page_url":"https://figshare.com/articles/dataset/Alteration_of_Gene_Expression_Profile_in_Niemann_Pick_Type_C_Mice_Correlates_with_Tissue_Damage_and_Oxidative_Stress/130216","pdf_url":null,"source":{"id":"https://openalex.org/S4377196282","display_name":"Figshare","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I4210132348","host_organization_name":"Figshare (United Kingdom)","host_organization_lineage":["https://openalex.org/I4210132348"],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"","raw_type":"Dataset"},{"id":"pmh:oai:localhost:10533/138283","doi":null,"is_oa":true,"landing_page_url":"http://hdl.handle.net/10533/138283","pdf_url":null,"source":{"id":"https://openalex.org/S4306402641","display_name":"LA Referencia (Red Federada de Repositorios Institucionales de Publicaciones Cient\u00edficas)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I4383465926","host_organization_name":"LA Referencia","host_organization_lineage":["https://openalex.org/I4383465926"],"host_organization_lineage_names":[],"type":"repository"},"license":"other-oa","license_id":"https://openalex.org/licenses/other-oa","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"PLOS ONE","raw_type":"info:eu-repo/semantics/article"},{"id":"pmh:oai:localhost:10533/138284","doi":null,"is_oa":true,"landing_page_url":"http://hdl.handle.net/10533/138284","pdf_url":null,"source":{"id":"https://openalex.org/S4306402641","display_name":"LA Referencia (Red Federada de Repositorios Institucionales de Publicaciones Cient\u00edficas)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I4383465926","host_organization_name":"LA Referencia","host_organization_lineage":["https://openalex.org/I4383465926"],"host_organization_lineage_names":[],"type":"repository"},"license":"other-oa","license_id":"https://openalex.org/licenses/other-oa","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"PLOS ONE","raw_type":"info:eu-repo/semantics/article"},{"id":"pmh:oai:localhost:10533/138285","doi":null,"is_oa":true,"landing_page_url":"http://hdl.handle.net/10533/138285","pdf_url":null,"source":{"id":"https://openalex.org/S4306402641","display_name":"LA Referencia (Red Federada de Repositorios Institucionales de Publicaciones Cient\u00edficas)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I4383465926","host_organization_name":"LA Referencia","host_organization_lineage":["https://openalex.org/I4383465926"],"host_organization_lineage_names":[],"type":"repository"},"license":"other-oa","license_id":"https://openalex.org/licenses/other-oa","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"PLOS ONE","raw_type":"info:eu-repo/semantics/article"},{"id":"pmh:oai:localhost:10533/238446","doi":null,"is_oa":true,"landing_page_url":"http://hdl.handle.net/10533/238446","pdf_url":null,"source":{"id":"https://openalex.org/S4306402641","display_name":"LA Referencia (Red Federada de Repositorios Institucionales de Publicaciones Cient\u00edficas)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I4383465926","host_organization_name":"LA Referencia","host_organization_lineage":["https://openalex.org/I4383465926"],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by-nc-nd","license_id":"https://openalex.org/licenses/cc-by-nc-nd","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"Plos One","raw_type":"info:eu-repo/semantics/article"},{"id":"pmh:oai:localhost:10533/241418","doi":null,"is_oa":true,"landing_page_url":"http://hdl.handle.net/10533/241418","pdf_url":null,"source":{"id":"https://openalex.org/S4306402641","display_name":"LA Referencia (Red Federada de Repositorios Institucionales de Publicaciones Cient\u00edficas)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I4383465926","host_organization_name":"LA Referencia","host_organization_lineage":["https://openalex.org/I4383465926"],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by-nc-nd","license_id":"https://openalex.org/licenses/cc-by-nc-nd","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"Plos One","raw_type":"info:eu-repo/semantics/article"},{"id":"pmh:oai:repositorio.uchile.cl:2250/165430","doi":null,"is_oa":true,"landing_page_url":"https://repositorio.uchile.cl/handle/2250/165430","pdf_url":null,"source":{"id":"https://openalex.org/S4306481413","display_name":"Universidad de Chile","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by-nc-nd","license_id":"https://openalex.org/licenses/cc-by-nc-nd","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"PLoS ONE","raw_type":"Art\u00edculo de revista"}],"best_oa_location":{"id":"doi:10.1371/journal.pone.0028777","doi":"https://doi.org/10.1371/journal.pone.0028777","is_oa":true,"landing_page_url":"https://doi.org/10.1371/journal.pone.0028777","pdf_url":"https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0028777&type=printable","source":{"id":"https://openalex.org/S202381698","display_name":"PLoS ONE","issn_l":"1932-6203","issn":["1932-6203"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["anvur-class-a","anvur-scientific","cwts-core","doaj","dongbi-c","doyens","erih-plus","jufo-1","ki-jl-1","medline","norway-1","poland-100","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310315706","host_organization_name":"Public Library of Science","host_organization_lineage":["https://openalex.org/P4310315706"],"host_organization_lineage_names":["Public Library of Science"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"PLoS ONE","raw_type":"journal-article"},"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","display_name":"Good health and well-being","score":0.9537000060081482}],"study_designs":[],"awards":[{"id":"https://openalex.org/G5574618443","display_name":null,"funder_award_id":"#15090007","funder_id":"https://openalex.org/F4320338206","funder_display_name":"Fondo de Financiamiento de Centros de Investigaci\u00f3n en \u00c1reas Prioritarias"}],"funders":[{"id":"https://openalex.org/F4320323458","display_name":"Universidad de Chile","ror":"https://ror.org/047gc3g35"},{"id":"https://openalex.org/F4320338206","display_name":"Fondo de Financiamiento de Centros de Investigaci\u00f3n en \u00c1reas Prioritarias","ror":null}],"has_content":{"pdf":true,"grobid_xml":true},"content_urls":{"pdf":"https://content.openalex.org/works/W2063467367.pdf","grobid_xml":"https://content.openalex.org/works/W2063467367.grobid-xml"},"referenced_works_count":53,"referenced_works":["https://openalex.org/W287983420","https://openalex.org/W758260955","https://openalex.org/W1561238117","https://openalex.org/W1577577364","https://openalex.org/W1605558459","https://openalex.org/W1749911542","https://openalex.org/W1871124898","https://openalex.org/W1900200896","https://openalex.org/W1968428170","https://openalex.org/W1971398322","https://openalex.org/W1982503072","https://openalex.org/W1985702872","https://openalex.org/W1990735027","https://openalex.org/W1996483226","https://openalex.org/W1999487819","https://openalex.org/W2007676659","https://openalex.org/W2011555635","https://openalex.org/W2015901846","https://openalex.org/W2020889176","https://openalex.org/W2022671309","https://openalex.org/W2035184644","https://openalex.org/W2035517268","https://openalex.org/W2040407461","https://openalex.org/W2045083711","https://openalex.org/W2050723884","https://openalex.org/W2058273503","https://openalex.org/W2059319138","https://openalex.org/W2064978465","https://openalex.org/W2066300584","https://openalex.org/W2068262899","https://openalex.org/W2070003798","https://openalex.org/W2070053386","https://openalex.org/W2079850786","https://openalex.org/W2081098333","https://openalex.org/W2087459059","https://openalex.org/W2105423509","https://openalex.org/W2108660588","https://openalex.org/W2109931172","https://openalex.org/W2110966376","https://openalex.org/W2117607208","https://openalex.org/W2120041926","https://openalex.org/W2121597422","https://openalex.org/W2124474318","https://openalex.org/W2128389187","https://openalex.org/W2129679875","https://openalex.org/W2136116905","https://openalex.org/W2137234884","https://openalex.org/W2138786573","https://openalex.org/W2149548143","https://openalex.org/W2168016965","https://openalex.org/W2169714384","https://openalex.org/W2169824691","https://openalex.org/W2320064004"],"related_works":["https://openalex.org/W7096195271","https://openalex.org/W2099423028","https://openalex.org/W2045083711","https://openalex.org/W2040407461","https://openalex.org/W4412637696","https://openalex.org/W2109931172","https://openalex.org/W2561348059","https://openalex.org/W2080982842","https://openalex.org/W2015901846","https://openalex.org/W2079203448"],"abstract_inverted_index":{"BACKGROUND:":[0],"Niemann-Pick":[1],"type":[2],"C":[3],"disease":[4,41,244],"(NPC)":[5],"is":[6,24,245,260],"a":[7,214],"neurovisceral":[8],"lipid":[9],"storage":[10,28],"disorder":[11],"mainly":[12,267],"characterized":[13,246],"by":[14,39,207,247],"unesterified":[15],"cholesterol":[16,124],"accumulation":[17],"in":[18,53,68,75,128,145,154,169,195,236,250,253,271,282,289],"lysosomal/late":[19],"endosomal":[20],"compartments,":[21],"although":[22],"there":[23],"also":[25,261],"an":[26,248],"important":[27],"for":[29],"several":[30],"other":[31],"kind":[32],"of":[33,96,111,132,165,182,222,268],"lipids.":[34],"The":[35],"main":[36],"tissues":[37],"affected":[38],"the":[40,43,46,69,71,108,146,290],"are":[42],"liver":[44,61,147,196,201,243],"and":[45,57,62,78,92,101,123,130,134,148,150,167,189,202,209,219,234,252,274],"cerebellum.":[47,63],"Oxidative":[48],"stress":[49,94,143,273],"has":[50,285],"been":[51,82,286],"described":[52],"various":[54,141],"NPC":[55,135,170,237,241,259,291],"cells":[56],"tissues,":[58],"such":[59],"as":[60,224,226,284],"Although":[64],"considerable":[65],"alterations":[66],"occur":[67],"liver,":[70],"pathological":[72],"mechanisms":[73],"involved":[74,270],"hepatocyte":[76],"damage":[77],"death":[79],"have":[80],"not":[81],"clearly":[83],"defined.":[84],"Here,":[85],"we":[86,199],"assessed":[87],"hepatic":[88,149,223],"tissue":[89,218],"integrity,":[90],"biochemical":[91],"oxidative":[93,117,142,231,257,272],"parameters":[95,144,281],"wild-type":[97],"control":[98,160],"(Npc1(+/+);":[99],"WT)":[100],"homozygous-mutant":[102],"(Npc1(-/-);":[103],"NPC)":[104],"mice.":[105,136,238],"In":[106,240],"addition,":[107],"mRNA":[109],"abundance":[110],"genes":[112,228,269],"encoding":[113],"proteins":[114],"associated":[115,229,255],"with":[116,159,179,230,256,263,279],"stress,":[118,232],"copper":[119,193],"metabolism,":[120],"fibrosis,":[121],"inflammation":[122,166,235],"metabolism":[125],"were":[126,177],"analyzed":[127,140,200],"livers":[129,171],"cerebella":[131],"WT":[133],"METHODOLOGY/PRINCIPAL":[137],"FINDINGS:":[138],"We":[139,162,212],"cerebellum":[151,203],"gene":[152,204,265],"expression":[153,205,221],"7-week-old":[155],"NPC1-deficient":[156],"mice":[157,292],"compared":[158],"animals.":[161],"found":[163,213],"signs":[164,176],"fibrosis":[168,233,251],"upon":[172],"histological":[173],"examination.":[174],"These":[175,276],"correlated":[178,262],"increased":[180,191],"levels":[181,194],"carbonylated":[183],"proteins,":[184],"diminished":[185],"total":[186,192],"glutathione":[187],"content":[188],"significantly":[190],"tissue.":[197],"Finally,":[198],"patterns":[206],"qPCR":[208],"microarray":[210],"assays.":[211],"correlation":[215],"between":[216],"fibrotic":[217],"differential":[220],"well":[225],"cerebellar":[227],"CONCLUSIONS/SIGNIFICANCE:":[239],"mice,":[242],"increase":[249],"markers":[254],"stress.":[258],"altered":[264],"expression,":[266],"fibrosis.":[275],"findings":[277],"correlate":[278],"similar":[280],"cerebellum,":[283],"previously":[287],"reported":[288],"model.":[293]},"counts_by_year":[{"year":2025,"cited_by_count":1},{"year":2023,"cited_by_count":1},{"year":2022,"cited_by_count":3},{"year":2021,"cited_by_count":4},{"year":2020,"cited_by_count":8},{"year":2019,"cited_by_count":4},{"year":2018,"cited_by_count":5},{"year":2017,"cited_by_count":6},{"year":2016,"cited_by_count":8},{"year":2015,"cited_by_count":4},{"year":2014,"cited_by_count":6},{"year":2013,"cited_by_count":6},{"year":2012,"cited_by_count":6}],"updated_date":"2026-11-10T11:39:43.021295","created_date":"2016-06-24T00:00:00"}