{"id":"https://openalex.org/W160958664","doi":"https://doi.org/10.1096/fasebj.26.1_supplement.1035.17","title":"Iron and copper in the pathogenesis of Friedreich's ataxia","display_name":"Iron and copper in the pathogenesis of Friedreich's ataxia","publication_year":2012,"publication_date":"2012-04-01","ids":{"openalex":"https://openalex.org/W160958664","doi":"https://doi.org/10.1096/fasebj.26.1_supplement.1035.17","mag":"160958664"},"language":"en","primary_location":{"id":"doi:10.1096/fasebj.26.1_supplement.1035.17","doi":"https://doi.org/10.1096/fasebj.26.1_supplement.1035.17","is_oa":false,"landing_page_url":"https://doi.org/10.1096/fasebj.26.1_supplement.1035.17","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","is_traditional":true,"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/A5043743871","display_name":"Joseph Edward Mazurkiewicz","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I4405260259","display_name":"Albany Medical College","ror":"https://ror.org/03g66yt05","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I4405259726","https://openalex.org/I4405260259"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Joseph Edward Mazurkiewicz","raw_affiliation_strings":["<!--<label><sup>1</sup></label>--> Center for Neuropharmacology and Neuroscience Albany Medical College Albany NY","1--> Center for Neuropharmacology and Neuroscience Albany Medical College Albany NY"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"<!--<label><sup>1</sup></label>--> Center for Neuropharmacology and Neuroscience Albany Medical College Albany NY","institution_ids":["https://openalex.org/I4405260259"]},{"raw_affiliation_string":"1--> Center for Neuropharmacology and Neuroscience Albany Medical College Albany NY","institution_ids":["https://openalex.org/I4405260259"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5062004674","display_name":"Arnulf H. Koeppen","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I4210109456","display_name":"Albany Stratton VA Medical Center Albany","ror":"https://ror.org/01rpj9v06","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1322918889","https://openalex.org/I2799886695","https://openalex.org/I4210094901","https://openalex.org/I4210109456"]},{"id":"https://openalex.org/I4405260259","display_name":"Albany Medical College","ror":"https://ror.org/03g66yt05","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I4405259726","https://openalex.org/I4405260259"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Arnulf H Koeppen","raw_affiliation_strings":["<!--<label><sup>2</sup></label>--> Neurology Albany Medical College Albany NY","<!--<label><sup>4</sup></label>--> Research Service VA Medical Center Albany NY","2--> Neurology Albany Medical College Albany NY","4--> Research Service VA Medical Center Albany NY"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"<!--<label><sup>2</sup></label>--> Neurology Albany Medical College Albany NY","institution_ids":["https://openalex.org/I4405260259"]},{"raw_affiliation_string":"<!--<label><sup>4</sup></label>--> Research Service VA Medical Center Albany NY","institution_ids":["https://openalex.org/I4210109456"]},{"raw_affiliation_string":"2--> Neurology Albany Medical College Albany NY","institution_ids":["https://openalex.org/I4405260259"]},{"raw_affiliation_string":"4--> Research Service VA Medical Center Albany NY","institution_ids":[]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5004954489","display_name":"Devin Yu","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I4210109456","display_name":"Albany Stratton VA Medical Center Albany","ror":"https://ror.org/01rpj9v06","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1322918889","https://openalex.org/I2799886695","https://openalex.org/I4210094901","https://openalex.org/I4210109456"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Devin Yu","raw_affiliation_strings":["<!--<label><sup>5</sup></label>--> VA Medical Center Albany NY","5--> VA Medical Center Albany NY"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"<!--<label><sup>5</sup></label>--> VA Medical Center Albany NY","institution_ids":["https://openalex.org/I4210109456"]},{"raw_affiliation_string":"5--> VA Medical Center Albany NY","institution_ids":["https://openalex.org/I4210109456"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5030783956","display_name":"Liane Ramirez","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I4210109456","display_name":"Albany Stratton VA Medical Center Albany","ror":"https://ror.org/01rpj9v06","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1322918889","https://openalex.org/I2799886695","https://openalex.org/I4210094901","https://openalex.org/I4210109456"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Liane Ramirez","raw_affiliation_strings":["<!--<label><sup>5</sup></label>--> VA Medical Center Albany NY","5--> VA Medical Center Albany NY"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"<!--<label><sup>5</sup></label>--> VA Medical Center Albany NY","institution_ids":["https://openalex.org/I4210109456"]},{"raw_affiliation_string":"5--> VA Medical Center Albany NY","institution_ids":["https://openalex.org/I4210109456"]}]},{"author_position":"middle","author":{"id":null,"display_name":"Jiang Qian","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I4405260259","display_name":"Albany Medical College","ror":"https://ror.org/03g66yt05","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I4405259726","https://openalex.org/I4405260259"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Jiang Qian","raw_affiliation_strings":["<!--<label><sup>3</sup></label>--> Pathology Albany Medical College Albany NY","3--> Pathology Albany Medical College Albany NY"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"<!--<label><sup>3</sup></label>--> Pathology Albany Medical College Albany NY","institution_ids":["https://openalex.org/I4405260259"]},{"raw_affiliation_string":"3--> Pathology Albany Medical College Albany NY","institution_ids":["https://openalex.org/I4405260259"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5061833203","display_name":"Patrick J. Parsons","orcid":"https://orcid.org/0000-0001-9133-875X","observed_orcids":["https://orcid.org/0000-0001-9133-875X"]},"institutions":[{"id":"https://openalex.org/I1339417872","display_name":"Wadsworth Center","ror":"https://ror.org/050kf9c55","country_code":"US","type":"facility","lineage":["https://openalex.org/I1298060332","https://openalex.org/I1339417872"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Patrick J Parsons","raw_affiliation_strings":["<!--<label><sup>6</sup></label>--> Wadsworth Center Albany NY","6--> Wadsworth Center Albany NY"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"<!--<label><sup>6</sup></label>--> Wadsworth Center Albany NY","institution_ids":["https://openalex.org/I1339417872"]},{"raw_affiliation_string":"6--> Wadsworth Center Albany NY","institution_ids":["https://openalex.org/I1339417872"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5028582386","display_name":"Karl X. Yang","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I1339417872","display_name":"Wadsworth Center","ror":"https://ror.org/050kf9c55","country_code":"US","type":"facility","lineage":["https://openalex.org/I1298060332","https://openalex.org/I1339417872"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Karl Yang","raw_affiliation_strings":["<!--<label><sup>6</sup></label>--> Wadsworth Center Albany NY","6--> Wadsworth Center Albany NY"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"<!--<label><sup>6</sup></label>--> Wadsworth Center Albany NY","institution_ids":["https://openalex.org/I1339417872"]},{"raw_affiliation_string":"6--> Wadsworth Center Albany NY","institution_ids":["https://openalex.org/I1339417872"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5065396969","display_name":"Zewu Chen","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Zewu Chen","raw_affiliation_strings":["<!--<label><sup>7</sup></label>--> X-Ray Optical Systems East Greenbush NY","7--> X-Ray Optical Systems East Greenbush NY"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"<!--<label><sup>7</sup></label>--> X-Ray Optical Systems East Greenbush NY","institution_ids":[]},{"raw_affiliation_string":"7--> X-Ray Optical Systems East Greenbush NY","institution_ids":[]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5000360560","display_name":"Paul J. Feustel","orcid":"https://orcid.org/0000-0002-6963-4349","observed_orcids":["https://orcid.org/0000-0002-6963-4349"]},"institutions":[{"id":"https://openalex.org/I4405260259","display_name":"Albany Medical College","ror":"https://ror.org/03g66yt05","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I4405259726","https://openalex.org/I4405260259"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Paul J Feustel","raw_affiliation_strings":["<!--<label><sup>1</sup></label>--> Center for Neuropharmacology and Neuroscience Albany Medical College Albany NY","1--> Center for Neuropharmacology and Neuroscience Albany Medical College Albany NY"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"<!--<label><sup>1</sup></label>--> Center for Neuropharmacology and Neuroscience Albany Medical College Albany NY","institution_ids":["https://openalex.org/I4405260259"]},{"raw_affiliation_string":"1--> Center for Neuropharmacology and Neuroscience Albany Medical College Albany NY","institution_ids":["https://openalex.org/I4405260259"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":3,"corresponding_author_ids":[],"corresponding_institution_ids":[],"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":0.03006418,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":null,"biblio":{"volume":"26","issue":"S1","first_page":null,"last_page":null},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10915","display_name":"Trace Elements in Health","score":0.4562999904155731,"subfield":{"id":"https://openalex.org/subfields/2916","display_name":"Nutrition and Dietetics"},"field":{"id":"https://openalex.org/fields/29","display_name":"Nursing"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},"topics":[{"id":"https://openalex.org/T10915","display_name":"Trace Elements in Health","score":0.4562999904155731,"subfield":{"id":"https://openalex.org/subfields/2916","display_name":"Nutrition and Dietetics"},"field":{"id":"https://openalex.org/fields/29","display_name":"Nursing"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T13481","display_name":"Neurological diseases and metabolism","score":0.4113999903202057,"subfield":{"id":"https://openalex.org/subfields/2808","display_name":"Neurology"},"field":{"id":"https://openalex.org/fields/28","display_name":"Neuroscience"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T10949","display_name":"Genetic Neurodegenerative Diseases","score":0.06350000202655792,"subfield":{"id":"https://openalex.org/subfields/2804","display_name":"Cellular and Molecular Neuroscience"},"field":{"id":"https://openalex.org/fields/28","display_name":"Neuroscience"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/friedreich-ataxia","display_name":"Friedreich's ataxia","score":1.0},{"id":"https://openalex.org/keywords/iron","display_name":"iron","score":0.7139999866485596},{"id":"https://openalex.org/keywords/copper","display_name":"copper","score":1.0},{"id":"https://openalex.org/keywords/zinc","display_name":"zinc","score":0.9190000295639038},{"id":"https://openalex.org/keywords/dentate-nucleus","display_name":"dentate nucleus","score":0.9829999804496765},{"id":"https://openalex.org/keywords/metal-distribution","display_name":"metal distribution","score":0.3109999895095825},{"id":"https://openalex.org/keywords/ferritin","display_name":"ferritin","score":0.8730000257492065},{"id":"https://openalex.org/keywords/oligodendroglia","display_name":"oligodendroglia","score":0.9819999933242798},{"id":"https://openalex.org/keywords/superoxide-dismutase","display_name":"superoxide dismutase","score":0.8930000066757202},{"id":"https://openalex.org/keywords/atp7a","display_name":"ATP7A","score":0.9079999923706055},{"id":"https://openalex.org/keywords/oxidative-damage","display_name":"oxidative damage","score":0.6380000114440918},{"id":"https://openalex.org/keywords/neurodegeneration","display_name":"neurodegeneration","score":0.7680000066757202},{"id":"https://openalex.org/keywords/x-ray-fluorescence-imaging","display_name":"X-ray fluorescence imaging","score":0.6389999985694885}],"concepts":[{"id":"https://openalex.org/C2778319317","wikidata":"https://www.wikidata.org/wiki/Q22327449","display_name":"Ferritin","level":2,"score":0.6792734265327454},{"id":"https://openalex.org/C2780906641","wikidata":"https://www.wikidata.org/wiki/Q213373","display_name":"Ataxia","level":2,"score":0.6724543571472168},{"id":"https://openalex.org/C2781192897","wikidata":"https://www.wikidata.org/wiki/Q822050","display_name":"White matter","level":3,"score":0.6095786094665527},{"id":"https://openalex.org/C2776710925","wikidata":"https://www.wikidata.org/wiki/Q24777245","display_name":"Ferroportin","level":4,"score":0.5748178362846375},{"id":"https://openalex.org/C185592680","wikidata":"https://www.wikidata.org/wiki/Q2329","display_name":"Chemistry","level":0,"score":0.508302628993988},{"id":"https://openalex.org/C2779314966","wikidata":"https://www.wikidata.org/wiki/Q409999","display_name":"Frataxin","level":4,"score":0.4645971953868866},{"id":"https://openalex.org/C2777106986","wikidata":"https://www.wikidata.org/wiki/Q1495005","display_name":"Copper deficiency","level":3,"score":0.4503992795944214},{"id":"https://openalex.org/C2776768029","wikidata":"https://www.wikidata.org/wiki/Q14882371","display_name":"Hepcidin","level":3,"score":0.43643882870674133},{"id":"https://openalex.org/C28452662","wikidata":"https://www.wikidata.org/wiki/Q422752","display_name":"Ceruloplasmin","level":2,"score":0.41715994477272034},{"id":"https://openalex.org/C142724271","wikidata":"https://www.wikidata.org/wiki/Q7208","display_name":"Pathology","level":1,"score":0.41033026576042175},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.3347601890563965},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.2809489965438843},{"id":"https://openalex.org/C2779369046","wikidata":"https://www.wikidata.org/wiki/Q6072234","display_name":"Iron-binding proteins","level":3,"score":0.24198955297470093},{"id":"https://openalex.org/C55493867","wikidata":"https://www.wikidata.org/wiki/Q7094","display_name":"Biochemistry","level":1,"score":0.23125207424163818},{"id":"https://openalex.org/C22814914","wikidata":"https://www.wikidata.org/wiki/Q410473","display_name":"Transferrin","level":2,"score":0.19838213920593262},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.17901387810707092},{"id":"https://openalex.org/C2778248108","wikidata":"https://www.wikidata.org/wiki/Q5445","display_name":"Anemia","level":2,"score":0.17678162455558777},{"id":"https://openalex.org/C544778455","wikidata":"https://www.wikidata.org/wiki/Q753","display_name":"Copper","level":2,"score":0.16249161958694458},{"id":"https://openalex.org/C169760540","wikidata":"https://www.wikidata.org/wiki/Q207011","display_name":"Neuroscience","level":1,"score":0.12756577134132385},{"id":"https://openalex.org/C143409427","wikidata":"https://www.wikidata.org/wiki/Q161238","display_name":"Magnetic resonance imaging","level":2,"score":0.10576316714286804},{"id":"https://openalex.org/C126838900","wikidata":"https://www.wikidata.org/wiki/Q77604","display_name":"Radiology","level":1,"score":0.0},{"id":"https://openalex.org/C178790620","wikidata":"https://www.wikidata.org/wiki/Q11351","display_name":"Organic chemistry","level":1,"score":0.0}],"mesh":[],"locations_count":1,"locations":[{"id":"doi:10.1096/fasebj.26.1_supplement.1035.17","doi":"https://doi.org/10.1096/fasebj.26.1_supplement.1035.17","is_oa":false,"landing_page_url":"https://doi.org/10.1096/fasebj.26.1_supplement.1035.17","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":[{"id":"https://openalex.org/study-designs/observational-study","display_name":"Observational Study"}],"awards":[],"funders":[{"id":"https://openalex.org/F4320307025","display_name":"National Ataxia Foundation","ror":"https://ror.org/03s9xxw55"}],"has_content":{"pdf":false,"grobid_xml":false},"content_urls":null,"referenced_works_count":0,"referenced_works":[],"related_works":["https://openalex.org/W2047228893","https://openalex.org/W7097639132","https://openalex.org/W4391030716","https://openalex.org/W1986984479","https://openalex.org/W2921245262","https://openalex.org/W2304630370","https://openalex.org/W2125771023","https://openalex.org/W2134102679","https://openalex.org/W1998617606","https://openalex.org/W2899929374"],"abstract_inverted_index":{"Friedreich's":[0],"ataxia":[1],"(FRDA)":[2],"causes":[3],"progressive":[4],"atrophy":[5],"of":[6,10,57,94,132,137,147,197],"the":[7,11,22,28,34,37,48,89,105,112,133,138,144,192,200],"large":[8],"neurons":[9],"dentate":[12],"nucleus":[13],"(DN).":[14],"Possible":[15],"contributing":[16],"factors":[17],"to":[18,32],"FRDA":[19,62,102,148,182],"pathogenesis":[20],"are":[21],"high":[23],"iron":[24],"(Fe)":[25],"concentration":[26],"in":[27,47,73,88,101,130,143,175,189],"DN":[29,49,145],"and":[30,43,52,64,77,81,154,163,177,219],"failure":[31],"clear":[33],"metal":[35,116],"from":[36,60,191],"damaged":[38],"tissue.":[39],"Fe,":[40],"copper":[41],"(Cu),":[42],"zinc":[44],"(Zn)":[45],"content":[46],"was":[50,129,173],"imaged":[51],"quantified":[53],"by":[54],"X\u2010ray":[55],"fluorescence":[56],"PEG\u2010embedded":[58],"tissue":[59],"10":[61],"patients":[63],"13":[65],"controls.":[66],"In":[67],"normal":[68,153,193],"DN,":[69,103],"Fe":[70,96],"occurred":[71],"predominantly":[72],"central":[74,134],"white":[75,135,202],"matter":[76,136,203],"hilus":[78,146],"whereas":[79],"Cu":[80,106,164,198],"Zn":[82],"were":[83,149],"present":[84,174],"at":[85],"peak":[86],"levels":[87],"gray":[90],"matter.":[91],"The":[92,121,195],"location":[93],"maximum":[95],"signal":[97],"did":[98,118,183],"not":[99,119,184],"change":[100,188],"but":[104,181],"region":[107],"broadened":[108],"overlapping":[109],"extensively":[110],"with":[111],"Fe\u2010rich":[113,201],"region.":[114],"Maximal":[115],"concentrations":[117],"change.":[120],"most":[122],"abundant":[123],"immunochemical":[124],"reaction":[125],"(ICR)":[126],"for":[127,158],"ferritin":[128],"oligodendroglia":[131,142],"DN.":[139],"Ferritin\u2010":[140],"reactive":[141],"much":[150],"smaller":[151],"than":[152],"more":[155],"numerous.":[156],"ICR":[157,190],"Cu,":[159],"Zn\u2010superoxide":[160],"dismutase":[161],"(SOD)":[162],"++":[165],"\u2010transporting":[166],"ATPase":[167],"\u03b1\u2010peptide":[168],"(ATP7A,":[169],"Menkes":[170],"disease":[171],"protein)":[172],"microglia":[176],"vessel":[178],"walls,":[179],"respectively,":[180],"cause":[185,205],"a":[186],"distinct":[187],"state.":[194],"shift":[196],"into":[199],"may":[204],"greater":[206],"oxidative":[207],"injury.":[208],"Supported":[209],"by:":[210],"NIH\u2010NS069454\u201002;":[211],"Friedreich\u02c8s":[212],"Ataxia":[213,217],"Research":[214],"Alliance;":[215],"National":[216],"Foundation;":[218],"Neurochemical":[220],"Research,":[221],"Inc":[222]},"counts_by_year":[],"updated_date":"2026-09-27T08:07:27.782135","created_date":"2016-06-24T00:00:00"}