{"id":"https://openalex.org/W4389023262","doi":"https://doi.org/10.1096/fasebj.31.1_supplement.1032.3","title":"Mitochondrial Metabolic Dysregulation and Structural Abnormalities in a Rat Model of Polycystic Kidney Disease","display_name":"Mitochondrial Metabolic Dysregulation and Structural Abnormalities in a Rat Model of Polycystic Kidney Disease","publication_year":2017,"publication_date":"2017-04-01","ids":{"openalex":"https://openalex.org/W4389023262","doi":"https://doi.org/10.1096/fasebj.31.1_supplement.1032.3"},"language":"en","primary_location":{"id":"doi:10.1096/fasebj.31.1_supplement.1032.3","doi":"https://doi.org/10.1096/fasebj.31.1_supplement.1032.3","is_oa":false,"landing_page_url":"https://doi.org/10.1096/fasebj.31.1_supplement.1032.3","pdf_url":null,"source":{"id":"https://openalex.org/S25293849","display_name":"The FASEB Journal","issn_l":"0892-6638","issn":["0892-6638","1530-6860"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-c","doyens","jufo-2","ki-jl-2","medline","norway-2","poland-140","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310320595","host_organization_name":"Wiley","host_organization_lineage":["https://openalex.org/P4310320595"],"host_organization_lineage_names":["Wiley"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"The FASEB Journal","raw_type":"journal-article"},"volume":null,"type":"article","indexed_in":["crossref"],"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/A5074327597","display_name":"Maria Valentina Irazabal","orcid":"https://orcid.org/0000-0002-9904-7778","observed_orcids":["https://orcid.org/0000-0002-9904-7778"]},"institutions":[{"id":"https://openalex.org/I1330342723","display_name":"Mayo Clinic","ror":"https://ror.org/02qp3tb03","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1330342723"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Maria V Irazabal","raw_affiliation_strings":["Nephrology and Hypertension, Mayo Clinic, Rochester, MN"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Nephrology and Hypertension, Mayo Clinic, Rochester, MN","institution_ids":["https://openalex.org/I1330342723"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5026864422","display_name":"Jennifer Arroyo","orcid":"https://orcid.org/0000-0001-8271-7184","observed_orcids":["https://orcid.org/0000-0001-8271-7184"]},"institutions":[{"id":"https://openalex.org/I1330342723","display_name":"Mayo Clinic","ror":"https://ror.org/02qp3tb03","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1330342723"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Jennifer Arroyo","raw_affiliation_strings":["Nephrology and Hypertension, Mayo Clinic, Rochester, MN"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Nephrology and Hypertension, Mayo Clinic, Rochester, MN","institution_ids":["https://openalex.org/I1330342723"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5073564494","display_name":"Ivan Vu\u010dkovi\u0107","orcid":"https://orcid.org/0000-0001-7806-7238","observed_orcids":["https://orcid.org/0000-0001-7806-7238"]},"institutions":[{"id":"https://openalex.org/I1330342723","display_name":"Mayo Clinic","ror":"https://ror.org/02qp3tb03","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1330342723"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Ivan Vuckovic","raw_affiliation_strings":["Metabolomics Core, Mayo Clinic, Rochester, MN"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Metabolomics Core, Mayo Clinic, Rochester, MN","institution_ids":["https://openalex.org/I1330342723"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5100709128","display_name":"Song Zhang","orcid":"https://orcid.org/0000-0003-0067-5780","observed_orcids":["https://orcid.org/0000-0003-0067-5780"]},"institutions":[{"id":"https://openalex.org/I1330342723","display_name":"Mayo Clinic","ror":"https://ror.org/02qp3tb03","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1330342723"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Song Zhang","raw_affiliation_strings":["Metabolomics Core, Mayo Clinic, Rochester, MN"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Metabolomics Core, Mayo Clinic, Rochester, MN","institution_ids":["https://openalex.org/I1330342723"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5042727507","display_name":"Petras P. Dzeja","orcid":"https://orcid.org/0000-0002-7014-9930","observed_orcids":["https://orcid.org/0000-0002-7014-9930"]},"institutions":[{"id":"https://openalex.org/I1330342723","display_name":"Mayo Clinic","ror":"https://ror.org/02qp3tb03","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1330342723"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Petras Dzeja","raw_affiliation_strings":["Metabolomics Core, Mayo Clinic, Rochester, MN"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Metabolomics Core, Mayo Clinic, Rochester, MN","institution_ids":["https://openalex.org/I1330342723"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5043681590","display_name":"Slobodan I Macura","orcid":"https://orcid.org/0000-0002-6054-5163","observed_orcids":["https://orcid.org/0000-0002-6054-5163"]},"institutions":[{"id":"https://openalex.org/I1330342723","display_name":"Mayo Clinic","ror":"https://ror.org/02qp3tb03","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1330342723"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Slobodan Macura","raw_affiliation_strings":["Metabolomics Core, Mayo Clinic, Rochester, MN"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Metabolomics Core, Mayo Clinic, Rochester, MN","institution_ids":["https://openalex.org/I1330342723"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5002238355","display_name":"Peter C. Harris","orcid":"https://orcid.org/0000-0002-5304-6593","observed_orcids":["https://orcid.org/0000-0002-5304-6593"]},"institutions":[{"id":"https://openalex.org/I1330342723","display_name":"Mayo Clinic","ror":"https://ror.org/02qp3tb03","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1330342723"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Peter Harris","raw_affiliation_strings":["Nephrology and Hypertension, Mayo Clinic, Rochester, MN"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Nephrology and Hypertension, Mayo Clinic, Rochester, MN","institution_ids":["https://openalex.org/I1330342723"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5070149489","display_name":"Vicente E. Torres","orcid":"https://orcid.org/0000-0003-2008-1576","observed_orcids":["https://orcid.org/0000-0003-2008-1576"]},"institutions":[{"id":"https://openalex.org/I1330342723","display_name":"Mayo Clinic","ror":"https://ror.org/02qp3tb03","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1330342723"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Vicente Torres","raw_affiliation_strings":["Nephrology and Hypertension, Mayo Clinic, Rochester, MN"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Nephrology and Hypertension, Mayo Clinic, Rochester, MN","institution_ids":["https://openalex.org/I1330342723"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":1,"corresponding_author_ids":[],"corresponding_institution_ids":["https://openalex.org/I1330342723"],"apc_list":{"value":3740,"currency":"USD","value_usd":3740},"apc_paid":null,"fwci":0.0,"has_fulltext":false,"cited_by_count":0,"citation_normalized_percentile":{"value":0.35505437,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":null,"biblio":{"volume":"31","issue":"S1","first_page":null,"last_page":null},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T11412","display_name":"Genetic and Kidney Cyst Diseases","score":0.9990000128746033,"subfield":{"id":"https://openalex.org/subfields/1311","display_name":"Genetics"},"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"}},"topics":[{"id":"https://openalex.org/T11412","display_name":"Genetic and Kidney Cyst Diseases","score":0.9990000128746033,"subfield":{"id":"https://openalex.org/subfields/1311","display_name":"Genetics"},"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/T12049","display_name":"Tuberous Sclerosis Complex Research","score":9.999999747378752e-05,"subfield":{"id":"https://openalex.org/subfields/2737","display_name":"Physiology"},"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/T11027","display_name":"Metabolism and Genetic Disorders","score":9.999999747378752e-05,"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/autosomal-dominant-polycystic-kidney-disease","display_name":"autosomal dominant polycystic kidney disease","score":0.949999988079071},{"id":"https://openalex.org/keywords/tca-cycle-metabolites","display_name":"TCA cycle metabolites","score":0.9190000295639038},{"id":"https://openalex.org/keywords/total-kidney-volume","display_name":"total kidney volume","score":0.8140000104904175},{"id":"https://openalex.org/keywords/mitochondrial-ultrastructure","display_name":"mitochondrial ultrastructure","score":0.6620000004768372},{"id":"https://openalex.org/keywords/proximal-tubule","display_name":"proximal tubule","score":0.6830000281333923},{"id":"https://openalex.org/keywords/collecting-duct","display_name":"collecting duct","score":0.8130000233650208},{"id":"https://openalex.org/keywords/citrate","display_name":"citrate","score":0.5759999752044678},{"id":"https://openalex.org/keywords/malate","display_name":"malate","score":0.6970000267028809},{"id":"https://openalex.org/keywords/fumarate","display_name":"fumarate","score":0.8939999938011169},{"id":"https://openalex.org/keywords/alpha-ketoglutarate","display_name":"alpha-ketoglutarate","score":0.875},{"id":"https://openalex.org/keywords/hippurate","display_name":"hippurate","score":0.9950000047683716},{"id":"https://openalex.org/keywords/metabolic-reprogramming","display_name":"metabolic reprogramming","score":0.6179999709129333}],"concepts":[{"id":"https://openalex.org/C2780145431","wikidata":"https://www.wikidata.org/wiki/Q15443105","display_name":"Polycystic kidney disease","level":3,"score":0.7763673663139343},{"id":"https://openalex.org/C134018914","wikidata":"https://www.wikidata.org/wiki/Q162606","display_name":"Endocrinology","level":1,"score":0.658906102180481},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.6302894353866577},{"id":"https://openalex.org/C2778653478","wikidata":"https://www.wikidata.org/wiki/Q1054718","display_name":"Kidney disease","level":2,"score":0.6181811690330505},{"id":"https://openalex.org/C2780091579","wikidata":"https://www.wikidata.org/wiki/Q9377","display_name":"Kidney","level":2,"score":0.6142095923423767},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.4775750935077667},{"id":"https://openalex.org/C77411442","wikidata":"https://www.wikidata.org/wiki/Q181100","display_name":"Urinary system","level":2,"score":0.46528521180152893},{"id":"https://openalex.org/C142724271","wikidata":"https://www.wikidata.org/wiki/Q7208","display_name":"Pathology","level":1,"score":0.3816036581993103},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.36146581172943115}],"mesh":[],"locations_count":1,"locations":[{"id":"doi:10.1096/fasebj.31.1_supplement.1032.3","doi":"https://doi.org/10.1096/fasebj.31.1_supplement.1032.3","is_oa":false,"landing_page_url":"https://doi.org/10.1096/fasebj.31.1_supplement.1032.3","pdf_url":null,"source":{"id":"https://openalex.org/S25293849","display_name":"The FASEB Journal","issn_l":"0892-6638","issn":["0892-6638","1530-6860"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-c","doyens","jufo-2","ki-jl-2","medline","norway-2","poland-140","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310320595","host_organization_name":"Wiley","host_organization_lineage":["https://openalex.org/P4310320595"],"host_organization_lineage_names":["Wiley"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"The FASEB Journal","raw_type":"journal-article"}],"best_oa_location":null,"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","score":0.9537000060081482,"display_name":"Good health and well-being"}],"study_designs":[],"awards":[],"funders":[],"has_content":{"pdf":false,"grobid_xml":false},"content_urls":null,"referenced_works_count":0,"referenced_works":[],"related_works":["https://openalex.org/W4397046339","https://openalex.org/W2994090564","https://openalex.org/W4206107451","https://openalex.org/W2887872978","https://openalex.org/W7160928467","https://openalex.org/W7112979508","https://openalex.org/W7170140537","https://openalex.org/W4396992113","https://openalex.org/W2782335474","https://openalex.org/W4413109639"],"abstract_inverted_index":{"Background":[0],"Autosomal":[1],"Dominant":[2],"Polycystic":[3,392],"Kidney":[4,162,393,410],"Disease":[5,394],"(PKD)":[6],"is":[7],"the":[8,15,24,45,59,66,71,110,209,243,270,341,355,389],"most":[9],"frequent":[10],"hereditary":[11],"renal":[12,21,166],"disease":[13,30,120,144,210],"and":[14,29,74,93,96,113,150,153,194,227,250,260,289,297,308,363,371,399,405],"fourth":[16],"leading":[17],"cause":[18],"of":[19,27,47,77,83,119,205,225,247,299,304,328],"end\u2010stage":[20],"disease.":[22],"However,":[23],"exact":[25],"mechanisms":[26],"cystogenesis":[28,64],"progression":[31],"remain":[32],"to":[33,52,86,108,142],"be":[34,53,367],"elucidated.":[35],"Recent":[36],"evidence":[37],"suggests":[38],"that":[39,360],"a":[40,123,217,239],"metabolic":[41,60,364],"reprograming":[42],"may":[43,366],"underlie":[44],"dysregulation":[46],"several":[48,329],"signaling":[49],"pathways":[50,61],"known":[51],"affected":[54],"in":[55,65,182,211,220,269,294,314,334,354,369,378],"PKD.":[56,379],"To":[57],"identify":[58],"associated":[62,349],"with":[63,101,116,173,200,284,340,350],"PKD":[67],"kidney,":[68],"we":[69],"characterized":[70],"urinary,":[72,151],"plasma,":[73,152],"tissue":[75,154,278],"metabolome":[76],"PCK":[78,95,212,226,300,335],"(a":[79],"well":[80],"established":[81],"model":[82],"PKD)":[84],"compared":[85],"Sprague\u2010Dawley":[87],"(SD)":[88],"wild\u2010type":[89],"rats.":[90],"Methods":[91],"Male":[92],"female":[94],"SD":[97,221,228],"rats":[98,213,222],"were":[99,146,171,292,312,348],"treated":[100],"dDAVP":[102,206],"(PCK\u2010D,":[103],"n=16":[104,130],"or":[105,126,131,381],"SD\u2010D,":[106],"n=12)":[107,133],"aggravate":[109],"cystic":[111,218,317],"phenotype":[112,219],"achieve":[114],"animals":[115],"different":[117],"degrees":[118],"severity":[121],"for":[122,409],"given":[124],"age,":[125],"saline":[127],"solution":[128],"(PCK\u2010S,":[129],"SD\u2010S,":[132],"as":[134,374],"controls.":[135],"Total":[136],"kidney":[137],"volume":[138],"(TKV)":[139,214],"gold":[140],"standard":[141],"assess":[143],"severity,":[145],"measured":[147],"by":[148,156,179,230,388],"MRI,":[149],"metabolites":[155,163,255,281,333],"proton":[157],"nuclear":[158],"magnetic":[159],"resonance,":[160],"1H\u2010NMR.":[161],"identified":[164],"from":[165,402],"tissues":[167],"collected":[168],"at":[169],"euthanasia":[170],"correlated":[172,282],"TKV.":[174],"Mitochondrial":[175,287],"structure":[176],"was":[177,386],"assessed":[178],"electron":[180],"microscopy":[181],"proximal":[183],"tubules":[184,192],"(PT),":[185],"medullary":[186],"thick":[187],"ascending":[188],"limb":[189],"(mTAL),":[190],"distal":[191],"(DT),":[193],"collecting":[195],"ducts":[196],"(CD).":[197],"Results":[198],"Consistent":[199],"previously":[201],"published":[202],"data,":[203],"administration":[204],"significantly":[207],"aggravated":[208,339],"without":[215],"inducing":[216],"().":[223,245,276,286,319],"Analysis":[224],"metabolomes":[229],"Partial":[231],"least":[232],"squares\u2010discriminant":[233],"analysis":[234,252,324],"(PLS\u2010DA)":[235],"score":[236],"plots":[237],"showed":[238,325],"significant":[240,326],"separation":[241],"between":[242],"groups":[244],"Integration":[246],"tissue,":[248],"urine":[249],"plasma":[251],"revealed":[253],"5":[254],"(\u03b1\u2010ketoglutarate,":[256],"fumarate,":[257],"malate,":[258],"citrate,":[259],"hippurate)":[261],"consistently":[262],"differentially":[263],"expressed,":[264],"among":[265],"which":[266,337],"4":[267],"participate":[268],"mitochondrial":[271,330,351,361],"tricarboxylic":[272],"acid":[273],"(TCA)":[274],"cycle":[275,280,332],"Moreover,":[277],"TCA":[279,331],"directly":[283],"TKV":[285],"area":[288],"matrix":[290,310],"density":[291,311],"preserved":[293],"PT,":[295],"mTAL,":[296],"DT":[298],"rats,":[301,336],"yet":[302],"disruption":[303],"cristae":[305],"membranes,":[306],"swelling,":[307],"decreased":[309],"observed":[313],"their":[315],"CD":[316],"cells":[318],"Conclusions":[320],"Comprehensive":[321],"multilevel":[322],"metabolomics":[323],"dysregulations":[327,365],"further":[338],"more":[342],"severe":[343],"phenotype.":[344],"Notably,":[345],"these":[346],"changes":[347],"structural":[352],"abnormalities":[353],"CD.":[356],"Our":[357],"observations":[358],"suggest":[359],"damage":[362],"implicated":[368],"cystogenesis,":[370],"position":[372],"mitochondria":[373],"important":[375],"therapeutic":[376],"targets":[377],"Support":[380],"Funding":[382],"Information":[383],"This":[384],"study":[385],"supported":[387],"Mayo":[390],"Translational":[391],"Center":[395],"(NIDDK":[396],"P30":[397],"DK090728)":[398],"an":[400],"endowment":[401],"Robert":[403],"M.":[404],"Billie":[406],"J.":[407],"Pirnie":[408],"Research":[411]},"counts_by_year":[],"updated_date":"2026-10-30T12:39:25.321706","created_date":"2023-11-27T00:00:00"}