{"id":"https://openalex.org/W1764237367","doi":null,"title":"Studies on the phenotype of LXRbeta-/- mice : From malabsorption to amyotrophic lateral sclerosis","display_name":"Studies on the phenotype of LXRbeta-/- mice : From malabsorption to amyotrophic lateral sclerosis","publication_year":2008,"publication_date":"2008-10-06","ids":{"openalex":"https://openalex.org/W1764237367","mag":"1764237367"},"language":"en","primary_location":{"id":"pmh:oai:figshare.com:article/26902684","doi":null,"is_oa":false,"landing_page_url":"https://figshare.com/articles/thesis/Studies_on_the_phenotype_of_LXR_-_-_mice_from_malabsorption_to_amyotrophic_lateral_sclerosis/26902684","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":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":true,"oa_status":"green","oa_url":"http://hdl.handle.net/10616/40179","any_repository_has_fulltext":true},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5012026877","display_name":"Chiara Gabbi","orcid":"https://orcid.org/0000-0002-9764-4771","observed_orcids":["https://orcid.org/0000-0002-9764-4771"]},"institutions":[{"id":"https://openalex.org/I28166907","display_name":"Karolinska Institutet","ror":"https://ror.org/056d84691","country_code":"SE","type":"education","lineage":["https://openalex.org/I28166907"]}],"countries":["SE"],"is_corresponding":true,"raw_author_name":"Chiara Gabbi","raw_affiliation_strings":["Karolinska Institutet"],"raw_orcid":"https://orcid.org/0000-0002-9764-4771","affiliations":[{"raw_affiliation_string":"Karolinska Institutet","institution_ids":["https://openalex.org/I28166907"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":1,"corresponding_author_ids":["https://openalex.org/A5012026877"],"corresponding_institution_ids":["https://openalex.org/I28166907"],"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/T10855","display_name":"Amyotrophic Lateral Sclerosis Research","score":0.6211000084877014,"subfield":{"id":"https://openalex.org/subfields/2728","display_name":"Clinical Neurology"},"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/T10855","display_name":"Amyotrophic Lateral Sclerosis Research","score":0.6211000084877014,"subfield":{"id":"https://openalex.org/subfields/2728","display_name":"Clinical Neurology"},"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":0.20409999787807465,"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/T10945","display_name":"Lysosomal Storage Disorders Research","score":0.022199999541044235,"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"}}],"keywords":[{"id":"https://openalex.org/keywords/liver-x-receptor","display_name":"liver X receptor","score":0.7710000276565552},{"id":"https://openalex.org/keywords/amyotrophic-lateral-sclerosis","display_name":"amyotrophic lateral sclerosis","score":0.9649999737739563},{"id":"https://openalex.org/keywords/motor-neuron-disease","display_name":"motor neuron disease","score":0.9750000238418579},{"id":"https://openalex.org/keywords/beta-sitosterol","display_name":"\u03b2-sitosterol","score":0.8930000066757202},{"id":"https://openalex.org/keywords/phytosterols","display_name":"phytosterols","score":0.7419999837875366},{"id":"https://openalex.org/keywords/abcg5-8","display_name":"ABCG5/8","score":0.7009999752044678},{"id":"https://openalex.org/keywords/npc1l1","display_name":"NPC1L1","score":0.8629999756813049},{"id":"https://openalex.org/keywords/cholesterol-transport","display_name":"cholesterol transport","score":0.46399998664855957},{"id":"https://openalex.org/keywords/intestinal-cholesterol-absorption","display_name":"intestinal cholesterol absorption","score":0.4480000138282776},{"id":"https://openalex.org/keywords/malabsorption","display_name":"malabsorption","score":0.5709999799728394},{"id":"https://openalex.org/keywords/tdp-43","display_name":"TDP-43","score":0.6639999747276306},{"id":"https://openalex.org/keywords/dopaminergic-neurons","display_name":"dopaminergic neurons","score":0.7129999995231628},{"id":"https://openalex.org/keywords/substantia-nigra","display_name":"substantia nigra","score":0.8399999737739563},{"id":"https://openalex.org/keywords/microglial-activation","display_name":"microglial activation","score":0.8240000009536743},{"id":"https://openalex.org/keywords/brain-cholesterol","display_name":"brain cholesterol","score":0.4180000126361847},{"id":"https://openalex.org/keywords/sphingomyelin","display_name":"sphingomyelin","score":0.5180000066757202},{"id":"https://openalex.org/keywords/ceramide","display_name":"ceramide","score":0.8650000095367432},{"id":"https://openalex.org/keywords/abcg-transporters","display_name":"ABCG transporters","score":0.5059999823570251},{"id":"https://openalex.org/keywords/lipid-metabolism","display_name":"lipid metabolism","score":0.4950000047683716}],"concepts":[{"id":"https://openalex.org/C2780596555","wikidata":"https://www.wikidata.org/wiki/Q206901","display_name":"Amyotrophic lateral sclerosis","level":3,"score":0.9369053840637207},{"id":"https://openalex.org/C127716648","wikidata":"https://www.wikidata.org/wiki/Q104053","display_name":"Phenotype","level":3,"score":0.6584327220916748},{"id":"https://openalex.org/C2780439177","wikidata":"https://www.wikidata.org/wiki/Q1442923","display_name":"Malabsorption","level":2,"score":0.6317178606987},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.5871663093566895},{"id":"https://openalex.org/C142724271","wikidata":"https://www.wikidata.org/wiki/Q7208","display_name":"Pathology","level":1,"score":0.3631317615509033},{"id":"https://openalex.org/C169760540","wikidata":"https://www.wikidata.org/wiki/Q207011","display_name":"Neuroscience","level":1,"score":0.3211013078689575},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.31199485063552856},{"id":"https://openalex.org/C54355233","wikidata":"https://www.wikidata.org/wiki/Q7162","display_name":"Genetics","level":1,"score":0.2641947865486145},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.23388317227363586},{"id":"https://openalex.org/C2779134260","wikidata":"https://www.wikidata.org/wiki/Q12136","display_name":"Disease","level":2,"score":0.20814216136932373},{"id":"https://openalex.org/C104317684","wikidata":"https://www.wikidata.org/wiki/Q7187","display_name":"Gene","level":2,"score":0.15262293815612793}],"mesh":[],"locations_count":3,"locations":[{"id":"pmh:oai:figshare.com:article/26902684","doi":null,"is_oa":false,"landing_page_url":"https://figshare.com/articles/thesis/Studies_on_the_phenotype_of_LXR_-_-_mice_from_malabsorption_to_amyotrophic_lateral_sclerosis/26902684","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":null,"license_id":null,"version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":null,"raw_type":"Thesis"},{"id":"pmh:oai:openarchive.ki.se:10616/40179","doi":null,"is_oa":true,"landing_page_url":"http://hdl.handle.net/10616/40179","pdf_url":null,"source":{"id":"https://openalex.org/S4306400147","display_name":"Open Archive (Karolinska Institutet)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I28166907","host_organization_name":"Karolinska Institutet","host_organization_lineage":["https://openalex.org/I28166907"],"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":"","raw_type":"info:eu-repo/semantics/other"},{"id":"mag:1764237367","doi":null,"is_oa":false,"landing_page_url":"https://openarchive.ki.se/xmlui/bitstream/10616/40179/1/thesis.pdf","pdf_url":null,"source":null,"license":null,"license_id":null,"version":null,"is_accepted":false,"is_published":false,"raw_source_name":null,"raw_type":null}],"best_oa_location":{"id":"pmh:oai:openarchive.ki.se:10616/40179","doi":null,"is_oa":true,"landing_page_url":"http://hdl.handle.net/10616/40179","pdf_url":null,"source":{"id":"https://openalex.org/S4306400147","display_name":"Open Archive (Karolinska Institutet)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I28166907","host_organization_name":"Karolinska Institutet","host_organization_lineage":["https://openalex.org/I28166907"],"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":"","raw_type":"info:eu-repo/semantics/other"},"sustainable_development_goals":[{"score":0.98580002784729,"display_name":"Good health and well-being","id":"https://metadata.un.org/sdg/3"}],"study_designs":[],"awards":[],"funders":[],"has_content":{"pdf":false,"grobid_xml":false},"content_urls":null,"referenced_works_count":91,"referenced_works":["https://openalex.org/W5897306","https://openalex.org/W1570388980","https://openalex.org/W1908881400","https://openalex.org/W1954999733","https://openalex.org/W1966283848","https://openalex.org/W1966343932","https://openalex.org/W1967286688","https://openalex.org/W1967983317","https://openalex.org/W1968963646","https://openalex.org/W1972009458","https://openalex.org/W1973243569","https://openalex.org/W1976830527","https://openalex.org/W1977873926","https://openalex.org/W1979674730","https://openalex.org/W1984284559","https://openalex.org/W1986945403","https://openalex.org/W1988692453","https://openalex.org/W1990437398","https://openalex.org/W1993211081","https://openalex.org/W1995533873","https://openalex.org/W1996350422","https://openalex.org/W2000592725","https://openalex.org/W2002245799","https://openalex.org/W2003798182","https://openalex.org/W2007478642","https://openalex.org/W2014020803","https://openalex.org/W2016022595","https://openalex.org/W2016493963","https://openalex.org/W2020780744","https://openalex.org/W2023011167","https://openalex.org/W2024679015","https://openalex.org/W2027140090","https://openalex.org/W2029354866","https://openalex.org/W2029475329","https://openalex.org/W2030442022","https://openalex.org/W2039774790","https://openalex.org/W2040417680","https://openalex.org/W2041781038","https://openalex.org/W2042035695","https://openalex.org/W2043834857","https://openalex.org/W2046658442","https://openalex.org/W2057519365","https://openalex.org/W2061725840","https://openalex.org/W2068201081","https://openalex.org/W2069532898","https://openalex.org/W2070395955","https://openalex.org/W2070582142","https://openalex.org/W2071470207","https://openalex.org/W2072251236","https://openalex.org/W2073228236","https://openalex.org/W2075788654","https://openalex.org/W2076452736","https://openalex.org/W2077083529","https://openalex.org/W2077671696","https://openalex.org/W2079080809","https://openalex.org/W2080254988","https://openalex.org/W2080305329","https://openalex.org/W2081630905","https://openalex.org/W2085909854","https://openalex.org/W2087863751","https://openalex.org/W2088364769","https://openalex.org/W2088436733","https://openalex.org/W2091277139","https://openalex.org/W2092193387","https://openalex.org/W2096386031","https://openalex.org/W2104016841","https://openalex.org/W2104606103","https://openalex.org/W2106636803","https://openalex.org/W2108928055","https://openalex.org/W2109839766","https://openalex.org/W2110437274","https://openalex.org/W2110904715","https://openalex.org/W2113624181","https://openalex.org/W2116495075","https://openalex.org/W2116613046","https://openalex.org/W2126276854","https://openalex.org/W2129972061","https://openalex.org/W2133182306","https://openalex.org/W2138651864","https://openalex.org/W2139936833","https://openalex.org/W2143615186","https://openalex.org/W2149457192","https://openalex.org/W2151255074","https://openalex.org/W2153526855","https://openalex.org/W2153673003","https://openalex.org/W2155925021","https://openalex.org/W2156259977","https://openalex.org/W2156575042","https://openalex.org/W2159228736","https://openalex.org/W2172108250","https://openalex.org/W2283034864"],"related_works":["https://openalex.org/W7015318178","https://openalex.org/W1583740721","https://openalex.org/W1894183494","https://openalex.org/W7033921806","https://openalex.org/W2116708889","https://openalex.org/W2063476471","https://openalex.org/W4414685373","https://openalex.org/W2462821056","https://openalex.org/W4385606460","https://openalex.org/W2149457192"],"abstract_inverted_index":{"Liver":[0],"X":[1,549],"Receptors,":[2],"LXRalpha":[3],"and":[4,27,36,46,73,157,166,233,244,261,283,293,302,351,422,429,453,557],"LXRbeta":[5,118,314,443,480,526],"(NR1H2)":[6],"are":[7,62,108,161,167,178,327],"nuclear":[8,42],"receptors":[9,43],"belonging":[10],"to":[11,64,120,196,335,362,392],"the":[12,23,32,98,112,126,141,170,182,220,226,230,235,249,265,298,311,330,345,360,402,405,420,431,440,445,470,491,506],"superfamily":[13],"of":[14,25,31,34,100,117,128,144,154,164,204,222,229,242,256,267,297,313,332,338,341,347,353,365,376,404,412,442,447,456,472,474,493,510,516,529],"ligand-activated":[15],"transcription":[16],"factors":[17],"with":[18,68,146,189,285,317,460,600],"a":[19,69,75,88,191,217,254,379,383,398,435,517,553,601],"key":[20],"role":[21,484],"in":[22,105,132,140,199,206,211,219,225,234,248,264,275,278,290,329,344,401,409,426,444,450,469,485,595,603],"control":[24],"lipid":[26],"glucose":[28],"metabolism.":[29],"Studies":[30],"phenotype":[33],"LXRalpha-/-mice":[35],"LXRbeta-/-mice":[37,61],"have":[38,44,74],"shown":[39,138],"that":[40,92,139,324,370,387,479],"these":[41,321,371],"specific":[45,436],"distinct":[47],"roles,":[48],"although":[49],"they":[50,86],"share":[51],"very":[52],"high":[53,70],"sequence":[54],"homology":[55],"(78%).":[56],"In":[57,84,181,246,319],"particular,":[58],"unlike":[59],"LXRalpha-/-mice,":[60],"resistant":[63],"obesity":[65],"when":[66],"fed":[67],"fat":[71,78],"diet":[72,82],"decreased":[76],"gonadal":[77],"pad":[79],"during":[80],"standard":[81,380],"feeding.":[83],"addition,":[85,247],"develop":[87],"motor":[89,129,223,237,349],"neuron":[90,130],"disease":[91,131],"resembles":[93],"amyotrophic":[94,147,558],"lateral":[95,148,559],"sclerosis":[96],"by":[97,173,310,454,499],"age":[99,377],"7":[101],"months.":[102],"The":[103,122,356],"studies":[104],"this":[106],"thesis":[107],"aimed":[109],"at":[110,373],"investigating":[111],"mechanisms":[113,326],"through":[114,490],"which":[115],"loss":[116,255,312],"leads":[119],"disease.":[121],"first":[123,183],"study":[124,184],"investigated":[125],"pathogenesis":[127],"LXRbeta-/-":[133,187,207,276,294,333,366,511,596],"mice.":[134,295,367],"It":[135],"has":[136],"been":[137],"spinal":[142,231],"cord":[143,232],"patients":[145,497],"sclerosis,":[149],"there":[150,215,239,252],"is":[151,416,598],"an":[152,262,461,467,482],"accumulation":[153,340],"sphingomyelin,":[155],"ceramides":[156],"cholesterol":[158,271,289],"esters:":[159],"phytosterols":[160],"known":[162,192],"ligands":[163],"LXRs":[165],"secreted":[168],"into":[169],"intestinal":[171,299],"lumen":[172],"ABCG":[174],"transporters.":[175],"These":[176,476],"transporters":[177],"LXR-regulated":[179],"genes.":[180],"we":[185,396,438,464],"treated":[186],"mice":[188,208,334,372,459,512,597],"beta-sitosterol,":[190],"motoneuron":[193],"toxin":[194],"thought":[195],"be":[197,514],"involved":[198,328],"ALS.":[200],"After":[201],"3":[202],"weeks":[203],"treatment,":[205],"but":[209],"not":[210,308],"wild":[212,280,457],"type":[213,281,458],"littermates,":[214],"was":[216,253],"reduction":[218,400,415,602],"number":[221,266],"neurons":[224,238,260],"lumbar":[227],"region":[228],"surviving":[236],"were":[240,273,307],"aggregates":[241,343],"ubiquitin":[243],"TDP-43.":[245],"substantia":[250],"nigra":[251],"tyrosine":[257],"hydroxylasepositive":[258],"dopaminergic":[259],"increase":[263,468],"activated":[268],"microglia.":[269],"Brain":[270],"concentrations":[272],"higher":[274],"than":[277],"their":[279,390],"counterparts,":[282],"treatment":[284,316,455],"beta-sitosterol":[286,556],"reduced":[287],"brain":[288,354],"both":[291],"WT":[292],"Levels":[296],"transporters,":[300],"ABCG5/8":[301],"Niemann-Pick":[303],"C1":[304],"Like":[305],"1,":[306],"affected":[309,498],"and/or":[315],"beta-sitosterol.":[318],"conclusion,":[320],"data":[322],"indicate":[323],"multiple":[325],"sensitivity":[331],"beta-sitosterol:":[336],"activation":[337],"microglia,":[339],"protein":[342],"cytoplasm":[346],"large":[348],"neurons,":[350],"depletion":[352],"cholesterol.":[355],"second":[357],"paper":[358],"examined":[359],"resistance":[361,391],"gain":[363,393],"weight":[364],"We":[368],"demonstrated":[369,439],"11":[374],"months":[375],"on":[378],"diet,":[381],"exhibit":[382,502],"pancreatic":[384,410,432,451,487],"exocrine":[385,503,593],"insufficiency":[386,594],"could":[388,465],"explain":[389],"weight.":[394],"Moreover,":[395],"showed":[397],"marked":[399],"expression":[403],"water":[406,494],"channel":[407],"aquaporin-1":[408,604],"ducts":[411,452],"LXRbeta-/-mice.":[413],"This":[414],"likely":[417],"responsible":[418],"for":[419,430],"thick":[421],"dense":[423],"secretion":[424,489],"visualized":[425],"electron":[427],"microscopy":[428],"insufficiency.":[433,504],"With":[434],"antibody":[437],"presence":[441],"nuclei":[446],"epithelial":[448],"cells":[449],"LXR":[462],"agonist":[463],"demonstrate":[466],"level":[471],"mRNA":[473],"aquaporin-1.":[475],"findings":[477],"suggest":[478],"plays":[481],"important":[483],"controlling":[486],"juice":[488],"regulation":[492],"transport.":[495],"Interestingly,":[496],"ALS":[500],"also":[501],"Thus":[505],"two":[507],"major":[508],"characteristics":[509],"may":[513],"part":[515],"single":[518],"syndrome":[519],"whose":[520],"common":[521],"etiology":[522],"stems":[523],"from":[524],"defective":[525],"signaling.":[527],"List":[528],"scientific":[530],"papers":[531],"I.":[532],"Kim":[533,579],"HJ,":[534,580],"Fan":[535],"X,":[536],"Gabbi":[537,577],"C,":[538,578],"Yakimchuk":[539],"K,":[540,582],"Parini":[541],"P,":[542],"Warner":[543,587],"M,":[544,588],"Gustafsson":[545,589],"JA":[546,590],"(2008).":[547,591],"\"Liver":[548],"receptor":[550],"beta":[551],"(LXRbeta):":[552],"link":[554],"between":[555],"sclerosis-Parkinsons":[560],"dementia.\"":[561],"Proc":[562,606],"Natl":[563,607],"Acad":[564,608],"Sci":[565,609],"U":[566,610],"S":[567,611],"A":[568,612],"105(6):":[569],"2094-9.":[570],"Epub":[571,615],"2008":[572,616],"Jan":[573],"31":[574],"https://pubmed.ncbi.nlm.nih.gov/18238900":[575],"II.":[576],"Hultenby":[581],"Bouton":[583],"D,":[584],"Toresson":[585],"G,":[586],"\"Pancreatic":[592],"associated":[599],"expression.\"":[605],"105(39):":[613],"15052-7.":[614],"Sep":[617],"19":[618],"https://pubmed.ncbi.nlm.nih.gov/18806227":[619]},"counts_by_year":[],"updated_date":"2026-11-08T12:12:28.789561","created_date":"2016-06-24T00:00:00"}
