{"id":"https://openalex.org/W7206358992","doi":null,"title":"THE ROLE OF NPC2 IN CHOLESTEROL TRANSPORT AND THE ENDOCYTIC SYSTEM IN NEUROBLASTOMA CELLS","display_name":"THE ROLE OF NPC2 IN CHOLESTEROL TRANSPORT AND THE ENDOCYTIC SYSTEM IN NEUROBLASTOMA CELLS","publication_year":2022,"publication_date":"2022-04-19","ids":{"openalex":"https://openalex.org/W7206358992"},"language":"en","primary_location":{"id":"pmh:oai:dal.scholaris.ca:10222/81622","doi":null,"is_oa":false,"landing_page_url":"http://hdl.handle.net/10222/81622","pdf_url":null,"source":{"id":"https://openalex.org/S7407064432","display_name":"DalSpace (Dalhousie University)","issn_l":"2041-2096","issn":["2041-2096"],"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":["dongbi-a","poland-140","russia-white-list-1"],"host_organization":"https://openalex.org/I129902397","host_organization_name":"Dalhousie University","host_organization_lineage":["https://openalex.org/I129902397"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":null,"raw_type":"Thesis"},"volume":null,"type":"dissertation","is_traditional":true,"indexed_in":[],"open_access":{"is_oa":false,"oa_status":"closed","oa_url":null,"any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5004321949","display_name":"Aaron Woblistin","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":true,"raw_author_name":"Woblistin, Aaron","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]}],"institutions":[],"countries_distinct_count":0,"institutions_distinct_count":0,"corresponding_author_ids":["https://openalex.org/A5004321949"],"corresponding_institution_ids":[],"apc_list":null,"apc_paid":null,"fwci":null,"has_fulltext":false,"cited_by_count":0,"citation_normalized_percentile":null,"cited_by_percentile_year":null,"biblio":{"volume":null,"issue":null,"first_page":null,"last_page":null},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10945","display_name":"Lysosomal Storage Disorders Research","score":0.9771000146865845,"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":0.9771000146865845,"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/T11618","display_name":"Cholesterol and Lipid Metabolism","score":0.014399999752640724,"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/T10617","display_name":"Cellular transport and secretion","score":0.0024999999441206455,"subfield":{"id":"https://openalex.org/subfields/1307","display_name":"Cell Biology"},"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/npc2","display_name":"NPC2","score":0.9919999837875366},{"id":"https://openalex.org/keywords/npc1","display_name":"NPC1","score":0.843999981880188},{"id":"https://openalex.org/keywords/niemann-pick-disease-type-c","display_name":"Niemann-Pick disease type C","score":0.9660000205039978},{"id":"https://openalex.org/keywords/cholesterol-transport","display_name":"cholesterol transport","score":0.9330000281333923},{"id":"https://openalex.org/keywords/late-endosomes","display_name":"late endosomes","score":0.7670000195503235},{"id":"https://openalex.org/keywords/lysosomal-cholesterol-accumulation","display_name":"lysosomal cholesterol accumulation","score":0.8289999961853027},{"id":"https://openalex.org/keywords/lysosomal-exocytosis","display_name":"lysosomal exocytosis","score":0.8460000157356262},{"id":"https://openalex.org/keywords/cholesterol-secretion","display_name":"cholesterol secretion","score":0.7590000033378601},{"id":"https://openalex.org/keywords/sh-sy5y-cells","display_name":"SH-SY5Y cells","score":0.8740000128746033}],"concepts":[{"id":"https://openalex.org/C2778552423","wikidata":"https://www.wikidata.org/wiki/Q14914279","display_name":"NPC1","level":4,"score":0.9455000162124634},{"id":"https://openalex.org/C2778163477","wikidata":"https://www.wikidata.org/wiki/Q43656","display_name":"Cholesterol","level":2,"score":0.8044999837875366},{"id":"https://openalex.org/C79747257","wikidata":"https://www.wikidata.org/wiki/Q189814","display_name":"Endocytic cycle","level":4,"score":0.7602999806404114},{"id":"https://openalex.org/C102747710","wikidata":"https://www.wikidata.org/wiki/Q824237","display_name":"Endosome","level":3,"score":0.7314000129699707},{"id":"https://openalex.org/C95444343","wikidata":"https://www.wikidata.org/wiki/Q7141","display_name":"Cell biology","level":1,"score":0.5906000137329102},{"id":"https://openalex.org/C185592680","wikidata":"https://www.wikidata.org/wiki/Q2329","display_name":"Chemistry","level":0,"score":0.5440999865531921},{"id":"https://openalex.org/C49039625","wikidata":"https://www.wikidata.org/wiki/Q84230","display_name":"Secretion","level":2,"score":0.4609000086784363},{"id":"https://openalex.org/C81885089","wikidata":"https://www.wikidata.org/wiki/Q189082","display_name":"Cell culture","level":2,"score":0.4528000056743622},{"id":"https://openalex.org/C28005876","wikidata":"https://www.wikidata.org/wiki/Q189814","display_name":"Endocytosis","level":3,"score":0.44909998774528503},{"id":"https://openalex.org/C110459506","wikidata":"https://www.wikidata.org/wiki/Q7318217","display_name":"Reverse cholesterol transport","level":4,"score":0.4392000138759613},{"id":"https://openalex.org/C175969161","wikidata":"https://www.wikidata.org/wiki/Q323426","display_name":"Exocytosis","level":3,"score":0.4311999976634979},{"id":"https://openalex.org/C130316041","wikidata":"https://www.wikidata.org/wiki/Q189206","display_name":"Vesicle","level":3,"score":0.4068000018596649},{"id":"https://openalex.org/C2777704780","wikidata":"https://www.wikidata.org/wiki/Q21115210","display_name":"ABCA1","level":4,"score":0.40380001068115234},{"id":"https://openalex.org/C79266657","wikidata":"https://www.wikidata.org/wiki/Q424178","display_name":"Lipid raft","level":3,"score":0.3815999925136566},{"id":"https://openalex.org/C2780216420","wikidata":"https://www.wikidata.org/wiki/Q83330","display_name":"Lysosome","level":3,"score":0.3815999925136566},{"id":"https://openalex.org/C134018914","wikidata":"https://www.wikidata.org/wiki/Q162606","display_name":"Endocrinology","level":1,"score":0.3522999882698059},{"id":"https://openalex.org/C2776925932","wikidata":"https://www.wikidata.org/wiki/Q1755122","display_name":"Neurodegeneration","level":3,"score":0.34220001101493835},{"id":"https://openalex.org/C139770010","wikidata":"https://www.wikidata.org/wiki/Q1339807","display_name":"Internalization","level":3,"score":0.336899995803833},{"id":"https://openalex.org/C2778106830","wikidata":"https://www.wikidata.org/wiki/Q29548","display_name":"Cell membrane","level":3,"score":0.33070001006126404},{"id":"https://openalex.org/C1491633281","wikidata":"https://www.wikidata.org/wiki/Q7868","display_name":"Cell","level":2,"score":0.3278999924659729},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.3084999918937683},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.30550000071525574},{"id":"https://openalex.org/C2779630084","wikidata":"https://www.wikidata.org/wiki/Q2073868","display_name":"Filipin","level":3,"score":0.30149999260902405},{"id":"https://openalex.org/C2779489717","wikidata":"https://www.wikidata.org/wiki/Q1419931","display_name":"Niemann\u2013Pick disease","level":3,"score":0.2937999963760376},{"id":"https://openalex.org/C189014844","wikidata":"https://www.wikidata.org/wiki/Q189118","display_name":"Cell type","level":3,"score":0.28040000796318054},{"id":"https://openalex.org/C55493867","wikidata":"https://www.wikidata.org/wiki/Q7094","display_name":"Biochemistry","level":1,"score":0.2766999900341034},{"id":"https://openalex.org/C54009773","wikidata":"https://www.wikidata.org/wiki/Q1429031","display_name":"Transfection","level":3,"score":0.2676999866962433},{"id":"https://openalex.org/C57947595","wikidata":"https://www.wikidata.org/wiki/Q7886088","display_name":"Membrane transport","level":3,"score":0.2660999894142151},{"id":"https://openalex.org/C70192387","wikidata":"https://www.wikidata.org/wiki/Q792602","display_name":"Axoplasmic transport","level":2,"score":0.25760000944137573},{"id":"https://openalex.org/C119062480","wikidata":"https://www.wikidata.org/wiki/Q83181","display_name":"Golgi apparatus","level":3,"score":0.2565999925136566}],"mesh":[],"locations_count":1,"locations":[{"id":"pmh:oai:dal.scholaris.ca:10222/81622","doi":null,"is_oa":false,"landing_page_url":"http://hdl.handle.net/10222/81622","pdf_url":null,"source":{"id":"https://openalex.org/S7407064432","display_name":"DalSpace (Dalhousie University)","issn_l":"2041-2096","issn":["2041-2096"],"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":["dongbi-a","poland-140","russia-white-list-1"],"host_organization":"https://openalex.org/I129902397","host_organization_name":"Dalhousie University","host_organization_lineage":["https://openalex.org/I129902397"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":null,"raw_type":"Thesis"}],"best_oa_location":null,"sustainable_development_goals":[{"score":0.9476000070571899,"display_name":"Good health and well-being","id":"https://metadata.un.org/sdg/3"}],"study_designs":[],"awards":[],"funders":[],"has_content":{"grobid_xml":false,"pdf":false},"content_urls":null,"referenced_works_count":0,"referenced_works":[],"related_works":["https://openalex.org/W1965969890","https://openalex.org/W1983014776","https://openalex.org/W2079745823","https://openalex.org/W2087412468","https://openalex.org/W1880941604","https://openalex.org/W2026717899","https://openalex.org/W2078583436","https://openalex.org/W2090606904","https://openalex.org/W2532270137","https://openalex.org/W82254553"],"abstract_inverted_index":{"Niemann-Pick":[0,16],"type":[1],"C":[2],"(NPC)":[3],"disease":[4,23,28],"is":[5,24,312],"an":[6],"autosomal":[7],"recessive,":[8],"neurodegenerative":[9],"disorder":[10],"caused":[11],"by":[12,30,194,271],"mutations":[13],"in":[14,35,53,106,123,133,162,176,201,217,232,236,257,303],"either":[15,97],"Type":[17],"C1":[18],"(NPC1)":[19],"or":[20,99,168,291,323],"NPC2.":[21],"NPC":[22],"a":[25,124,186,195,254,299],"lysosomal":[26,199],"lipid-storage":[27],"characterized":[29],"accumulation":[31,122],"of":[32,87,96,104,147,221,250,262,274,279,301,309,318,336,348],"unesterified":[33],"cholesterol":[34,46,73,81,89,105,121,149,158,306,322,345],"late":[36,228],"endosomes/lysosomes":[37],"(LE/L).":[38],"NPC2,":[39],"located":[40],"to":[41,51,58,61,102,119,131,150,154,166,207,239,285,305,320],"the":[42,54,62,66,85,136,151,218,248,266,316,334,339,344],"LE/L":[43,55,120],"lumen,":[44],"binds":[45],"and":[47,65,75,178,204,227,293],"hands":[48],"it":[49],"off":[50],"NPC1":[52,98],"perimeter":[56],"membrane":[57,64],"be":[59],"presented":[60],"plasma":[63],"ER.":[67],"Mature":[68],"neurons":[69],"do":[70],"not":[71,115,313],"synthesize":[72],"efficiently":[74],"rely":[76],"on":[77,265,315],"uptake":[78],"for":[79,198],"their":[80,258],"needs,":[82],"thereby":[83],"highlighting":[84],"importance":[86,335],"this":[88],"transport":[90,146,307,346],"pathway.":[91],"In":[92,242],"human":[93,125],"fibroblasts,":[94],"depletion":[95,117,264],"NPC2":[100,116,263,276,280,302,319,337],"leads":[101],"sequestration":[103],"LE/L.":[107],"Here,":[108],"I":[109],"show":[110],"that":[111,282],"only":[112],"NPC1,":[113,287,326],"but":[114,234,252,277],"led":[118],"neuroblastoma":[126],"cell":[127],"line":[128],"(SH-SY5Y),":[129],"contrary":[130],"results":[132,297],"fibroblasts":[134],"using":[135],"same":[137],"RNA":[138],"interference":[139],"system.":[140],"NPC2-depleted":[141,163,179,205,222],"cells":[142,164,184,246],"still":[143],"showed":[144,253],"impaired":[145],"endocytosed":[148],"ER,":[152],"similarly":[153],"NPC1-depleted":[155,169,244],"cells.":[156,170,210,241],"Cellular":[157],"levels":[159],"were":[160,215,230],"lower":[161],"compared":[165,206,238],"control":[167,208,240],"Cholesterol":[171],"secretion":[172],"was":[173,192,269],"markedly":[174],"reduced":[175,231],"NPC1-":[177,203],"SH-SY5Y":[180,209,223,245],"cells,":[181,224],"with":[182,325],"NPC2-deficient":[183],"showing":[185],"more":[187],"severe":[188],"reduction.":[189],"This":[190,260],"reduction":[191],"accompanied":[193],"decreased":[196],"capacity":[197],"exocytosis":[200],"both":[202],"The":[211],"most":[212],"striking":[213],"abnormalities":[214],"seen":[216],"endocytic":[219,267],"system":[220,268],"where":[225],"early":[226],"endosomes":[229],"number":[233,249],"similar":[235],"size":[237],"contrast,":[243],"maintained":[247],"vesicles":[251],"significant":[255],"increase":[256],"size.":[259],"effect":[261],"rescued":[270],"simultaneous":[272],"expression":[273],"wildtype":[275],"also":[278],"variants":[281],"are":[283,288],"unable":[284],"bind":[286,321],"sterol":[289],"binding-deficient,":[290],"glycosylation-":[292],"cellular":[294],"retention-deficient.":[295],"These":[296,331],"suggest":[298],"role":[300,341],"addition":[304],"out":[308],"LE/L,":[310],"which":[311],"dependent":[314],"ability":[317],"interact":[324],"potentially":[327],"regulating":[328],"endosome":[329],"homeostasis.":[330],"findings":[332],"highlight":[333],"outside":[338],"dogmatic":[340],"as":[342],"merely":[343],"partner":[347],"NPC1.":[349]},"counts_by_year":[],"updated_date":"2026-11-04T10:54:05.112534","created_date":"2026-09-03T00:00:00"}
