{"id":"https://openalex.org/W1921375299","doi":null,"title":"functional analyses of variants of human SCO1, a mitochondrial metallochaperone","display_name":"functional analyses of variants of human SCO1, a mitochondrial metallochaperone","publication_year":2013,"publication_date":"2013-10-17","ids":{"openalex":"https://openalex.org/W1921375299","mag":"1921375299"},"language":"en","primary_location":{"id":"pmh:oai:ecommons.usask.ca:10388/ETD-2013-09-1245","doi":null,"is_oa":false,"landing_page_url":"http://hdl.handle.net/10388/ETD-2013-09-1245","pdf_url":null,"source":{"id":"https://openalex.org/S4377196608","display_name":"University Library - University of Saskatchewan (University of Saskatchewan)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I32625721","host_organization_name":"University of Saskatchewan","host_organization_lineage":["https://openalex.org/I32625721"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"","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":null,"display_name":"Min Pan","orcid":"https://orcid.org/0000-0002-9781-0637","observed_orcids":["https://orcid.org/0000-0002-9781-0637"]},"institutions":[],"countries":[],"is_corresponding":true,"raw_author_name":"Min Pan","raw_affiliation_strings":[],"raw_orcid":"https://orcid.org/0000-0002-9781-0637","affiliations":[]}],"institutions":[],"countries_distinct_count":0,"institutions_distinct_count":0,"corresponding_author_ids":[],"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/T10301","display_name":"Mitochondrial Function and Pathology","score":0.9740999937057495,"subfield":{"id":"https://openalex.org/subfields/1312","display_name":"Molecular 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"}},"topics":[{"id":"https://openalex.org/T10301","display_name":"Mitochondrial Function and Pathology","score":0.9740999937057495,"subfield":{"id":"https://openalex.org/subfields/1312","display_name":"Molecular 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"}},{"id":"https://openalex.org/T11027","display_name":"Metabolism and Genetic Disorders","score":0.005400000140070915,"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"}},{"id":"https://openalex.org/T11747","display_name":"Metal-Catalyzed Oxygenation Mechanisms","score":0.004000000189989805,"subfield":{"id":"https://openalex.org/subfields/1604","display_name":"Inorganic Chemistry"},"field":{"id":"https://openalex.org/fields/16","display_name":"Chemistry"},"domain":{"id":"https://openalex.org/domains/3","display_name":"Physical Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/sco1","display_name":"SCO1","score":0.9860000014305115},{"id":"https://openalex.org/keywords/cytochrome-c-oxidase","display_name":"cytochrome c oxidase","score":0.8169999718666077},{"id":"https://openalex.org/keywords/copper-binding-proteins","display_name":"copper-binding proteins","score":0.5149999856948853},{"id":"https://openalex.org/keywords/metallochaperone","display_name":"metallochaperone","score":0.8119999766349792},{"id":"https://openalex.org/keywords/copper-transfer","display_name":"copper transfer","score":0.4169999957084656},{"id":"https://openalex.org/keywords/cua-site","display_name":"CuA site","score":0.8640000224113464},{"id":"https://openalex.org/keywords/cysteine-residues","display_name":"cysteine residues","score":0.7440000176429749},{"id":"https://openalex.org/keywords/conserved-histidine","display_name":"conserved histidine","score":0.8579999804496765},{"id":"https://openalex.org/keywords/copper-efflux","display_name":"copper efflux","score":0.6150000095367432},{"id":"https://openalex.org/keywords/fibroblasts","display_name":"fibroblasts","score":0.6909999847412109}],"concepts":[{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.36527007818222046},{"id":"https://openalex.org/C70721500","wikidata":"https://www.wikidata.org/wiki/Q177005","display_name":"Computational biology","level":1,"score":0.3284638226032257}],"mesh":[],"locations_count":3,"locations":[{"id":"pmh:oai:ecommons.usask.ca:10388/ETD-2013-09-1245","doi":null,"is_oa":false,"landing_page_url":"http://hdl.handle.net/10388/ETD-2013-09-1245","pdf_url":null,"source":{"id":"https://openalex.org/S4377196608","display_name":"University Library - University of Saskatchewan (University of Saskatchewan)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I32625721","host_organization_name":"University of Saskatchewan","host_organization_lineage":["https://openalex.org/I32625721"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"","raw_type":"thesis"},{"id":"pmh:oai:harvest.usask.ca:10388/ETD-2013-09-1245","doi":null,"is_oa":false,"landing_page_url":"https://hdl.handle.net/10388/ETD-2013-09-1245","pdf_url":null,"source":{"id":"https://openalex.org/S7407063267","display_name":"HARVEST (University of Saskatchewan)","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":null,"license_id":null,"version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":null,"raw_type":"Thesis"},{"id":"mag:1921375299","doi":null,"is_oa":false,"landing_page_url":"https://harvest.usask.ca/bitstream/10388/ETD-2013-09-1245/3/PAN-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":null,"sustainable_development_goals":[{"score":0.4043000042438507,"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":233,"referenced_works":["https://openalex.org/W55968684","https://openalex.org/W78512322","https://openalex.org/W123675027","https://openalex.org/W162377261","https://openalex.org/W174320801","https://openalex.org/W191693931","https://openalex.org/W1477052822","https://openalex.org/W1478988028","https://openalex.org/W1481324524","https://openalex.org/W1487824094","https://openalex.org/W1507935478","https://openalex.org/W1516459565","https://openalex.org/W1522064584","https://openalex.org/W1532982760","https://openalex.org/W1551444218","https://openalex.org/W1551504358","https://openalex.org/W1573818513","https://openalex.org/W1576746816","https://openalex.org/W1596172840","https://openalex.org/W1597688727","https://openalex.org/W1607363557","https://openalex.org/W1676079444","https://openalex.org/W1793487593","https://openalex.org/W1928774984","https://openalex.org/W1937544647","https://openalex.org/W1963874808","https://openalex.org/W1966686696","https://openalex.org/W1967888351","https://openalex.org/W1968048466","https://openalex.org/W1968408636","https://openalex.org/W1969825525","https://openalex.org/W1971442726","https://openalex.org/W1972343164","https://openalex.org/W1973925468","https://openalex.org/W1974003496","https://openalex.org/W1974166172","https://openalex.org/W1974260422","https://openalex.org/W1974572661","https://openalex.org/W1975927777","https://openalex.org/W1976157614","https://openalex.org/W1976535783","https://openalex.org/W1978370714","https://openalex.org/W1979008056","https://openalex.org/W1983520552","https://openalex.org/W1983834133","https://openalex.org/W1984164475","https://openalex.org/W1984788563","https://openalex.org/W1985609571","https://openalex.org/W1986514138","https://openalex.org/W1987630668","https://openalex.org/W1989026904","https://openalex.org/W1989705623","https://openalex.org/W1990357564","https://openalex.org/W1990529395","https://openalex.org/W1991020250","https://openalex.org/W1991485583","https://openalex.org/W1994335807","https://openalex.org/W1995095129","https://openalex.org/W1995360843","https://openalex.org/W1996676050","https://openalex.org/W1996715099","https://openalex.org/W2000523097","https://openalex.org/W2001173613","https://openalex.org/W2001754324","https://openalex.org/W2002364392","https://openalex.org/W2003033163","https://openalex.org/W2003152730","https://openalex.org/W2003562213","https://openalex.org/W2003717466","https://openalex.org/W2005221933","https://openalex.org/W2006159932","https://openalex.org/W2007195150","https://openalex.org/W2007354562","https://openalex.org/W2009064016","https://openalex.org/W2010526388","https://openalex.org/W2010588708","https://openalex.org/W2011995208","https://openalex.org/W2012005944","https://openalex.org/W2012560500","https://openalex.org/W2013504014","https://openalex.org/W2014112152","https://openalex.org/W2015391531","https://openalex.org/W2015588708","https://openalex.org/W2016135200","https://openalex.org/W2019011355","https://openalex.org/W2019322413","https://openalex.org/W2019913401","https://openalex.org/W2020332723","https://openalex.org/W2022522930","https://openalex.org/W2025364264","https://openalex.org/W2025753620","https://openalex.org/W2026869749","https://openalex.org/W2029342749","https://openalex.org/W2030996319","https://openalex.org/W2031331113","https://openalex.org/W2032559663","https://openalex.org/W2033538725","https://openalex.org/W2034621386","https://openalex.org/W2036794157","https://openalex.org/W2037255510","https://openalex.org/W2037776197","https://openalex.org/W2038219691","https://openalex.org/W2038487855","https://openalex.org/W2038770351","https://openalex.org/W2038976751","https://openalex.org/W2039168627","https://openalex.org/W2040582519","https://openalex.org/W2041359223","https://openalex.org/W2041499605","https://openalex.org/W2042465411","https://openalex.org/W2042774137","https://openalex.org/W2043529892","https://openalex.org/W2044038144","https://openalex.org/W2045303124","https://openalex.org/W2049043816","https://openalex.org/W2049316736","https://openalex.org/W2050729907","https://openalex.org/W2050796659","https://openalex.org/W2051891646","https://openalex.org/W2052322256","https://openalex.org/W2052563837","https://openalex.org/W2052595824","https://openalex.org/W2052720730","https://openalex.org/W2054006271","https://openalex.org/W2057566034","https://openalex.org/W2058697823","https://openalex.org/W2062167511","https://openalex.org/W2062884915","https://openalex.org/W2063006050","https://openalex.org/W2064847009","https://openalex.org/W2066236740","https://openalex.org/W2066867262","https://openalex.org/W2069021425","https://openalex.org/W2069176557","https://openalex.org/W2070622658","https://openalex.org/W2071030118","https://openalex.org/W2071570350","https://openalex.org/W2071656309","https://openalex.org/W2072588550","https://openalex.org/W2073888189","https://openalex.org/W2077574127","https://openalex.org/W2077617405","https://openalex.org/W2079043320","https://openalex.org/W2079281555","https://openalex.org/W2084034472","https://openalex.org/W2084974680","https://openalex.org/W2086407927","https://openalex.org/W2087514312","https://openalex.org/W2087813537","https://openalex.org/W2088656604","https://openalex.org/W2089407686","https://openalex.org/W2090528992","https://openalex.org/W2092460504","https://openalex.org/W2093319492","https://openalex.org/W2093545955","https://openalex.org/W2093785402","https://openalex.org/W2094539501","https://openalex.org/W2094617289","https://openalex.org/W2097771231","https://openalex.org/W2098440339","https://openalex.org/W2098842133","https://openalex.org/W2101117598","https://openalex.org/W2105532922","https://openalex.org/W2109291482","https://openalex.org/W2109415448","https://openalex.org/W2110205863","https://openalex.org/W2111375801","https://openalex.org/W2113015426","https://openalex.org/W2114341896","https://openalex.org/W2114673327","https://openalex.org/W2116034532","https://openalex.org/W2116562304","https://openalex.org/W2117488437","https://openalex.org/W2119221964","https://openalex.org/W2120587654","https://openalex.org/W2120741711","https://openalex.org/W2120763876","https://openalex.org/W2121061037","https://openalex.org/W2121117469","https://openalex.org/W2121198137","https://openalex.org/W2124287644","https://openalex.org/W2125861112","https://openalex.org/W2126388877","https://openalex.org/W2127901454","https://openalex.org/W2128130421","https://openalex.org/W2128635872","https://openalex.org/W2131143886","https://openalex.org/W2132254933","https://openalex.org/W2133033875","https://openalex.org/W2135248938","https://openalex.org/W2137051822","https://openalex.org/W2137158408","https://openalex.org/W2137247539","https://openalex.org/W2138781515","https://openalex.org/W2138881473","https://openalex.org/W2140751228","https://openalex.org/W2141002664","https://openalex.org/W2141163376","https://openalex.org/W2144287696","https://openalex.org/W2144444019","https://openalex.org/W2147409552","https://openalex.org/W2150164439","https://openalex.org/W2150264392","https://openalex.org/W2151234721","https://openalex.org/W2151271025","https://openalex.org/W2152004311","https://openalex.org/W2157062918","https://openalex.org/W2157064216","https://openalex.org/W2157068598","https://openalex.org/W2157334943","https://openalex.org/W2158356403","https://openalex.org/W2158642235","https://openalex.org/W2158742931","https://openalex.org/W2158881247","https://openalex.org/W2159403505","https://openalex.org/W2160226542","https://openalex.org/W2160992842","https://openalex.org/W2161291398","https://openalex.org/W2161407287","https://openalex.org/W2163811645","https://openalex.org/W2166436849","https://openalex.org/W2166568681","https://openalex.org/W2167586962","https://openalex.org/W2168165481","https://openalex.org/W2170076251","https://openalex.org/W2170407368","https://openalex.org/W2171628789","https://openalex.org/W2173353469","https://openalex.org/W2220950257","https://openalex.org/W2226416434","https://openalex.org/W2318413400","https://openalex.org/W2328092139","https://openalex.org/W3025805620"],"related_works":["https://openalex.org/W2133033875","https://openalex.org/W1997819934","https://openalex.org/W2120587654","https://openalex.org/W1984164475","https://openalex.org/W1996676050","https://openalex.org/W2533908340","https://openalex.org/W2079043320","https://openalex.org/W7097809978","https://openalex.org/W2087813537","https://openalex.org/W2157068598"],"abstract_inverted_index":{"Cytochrome":[0,28],"c":[1,29],"oxidase":[2],"(COX)":[3],"is":[4,95,116],"a":[5,53,89,98,108,112,206],"multimeric":[6],"protein":[7,124,179,249],"complex":[8],"whose":[9],"enzymatic":[10],"activity":[11,259],"contributes":[12],"to":[13,21,45,78,141,144,172,177,185,197],"the":[14,38,79,119,123,128,163,166,170,187,194,198,234,238,267,287,294,317,322,332,338,341],"generation":[15],"of":[16,27,37,50,67,122,159,169,189,201,208,243,252,270,276,289,297,321,331,335,340,344],"an":[17,156],"electrochemical":[18],"potential":[19],"required":[20,44,247],"synthesize":[22],"adenosine":[23],"triphosphate":[24],"(ATP).":[25],"Synthesis":[26],"Oxidase":[30],"1":[31],"(SCO1)":[32],"and":[33,71,87,107,125,147,152,174,193,210,220,223,282,286,293,299],"SCO2":[34,72,137],"are":[35,43,73,245],"two":[36,235],"many":[39],"accessory":[40],"factors":[41],"that":[42,233,307,327],"assemble":[46],"individual":[47],"structural":[48],"subunits":[49],"COX":[51,145,229,258,345],"into":[52,127,218],"functional":[54],"holoenzyme":[55],"complex.":[56],"Mutations":[57,132],"in":[58,118,133,155,260,337],"either":[59,134],"SCO":[60,178],"gene":[61],"cause":[62],"severe,":[63],"early":[64],"onset":[65],"forms":[66,275],"human":[68],"disease.":[69],"SCO1":[70,135,212,244,254,278,310,323,336],"closely":[74],"related":[75],"paralogues":[76],"localized":[77],"inner":[80],"mitochondrial":[81,129],"membrane.":[82],"Both":[83],"proteins":[84,214],"bind":[85,173],"copper":[86,143,160,176,292],"exhibit":[88],"thiol":[90],"disulphide":[91],"oxidoreductase":[92],"activity.":[93,230],"Copper":[94],"bound":[96,291],"by":[97,215],"highly":[99],"conserved":[100,195],"Cysteine":[101,105],"x":[102,103,104],"motif":[106],"histidine":[109,196],"found":[110,232],"within":[111,237],"thioredoxin":[113],"fold,":[114],"which":[115],"contained":[117],"C-terminal":[120,211,253,272],"half":[121],"projects":[126],"intermembrane":[130],"space.":[131],"or":[136],"affect":[138],"their":[139,225],"ability":[140,171],"deliver":[142],"II":[146],"metallate":[148],"its":[149],"CuA":[150,342],"site,":[151],"also":[153],"result":[154],"increased":[157],"rate":[158],"efflux":[161],"from":[162,284],"cell.":[164],"However,":[165],"relative":[167,295],"importance":[168],"transfer":[175],"function":[180],"remains":[181],"poorly":[182],"understood.":[183],"Therefore,":[184],"investigate":[186],"significance":[188],"several":[190],"cysteine":[191],"residues":[192],"copper-binding":[199,268],"properties":[200,269,326],"SCO1,":[202],"I":[203,231],"functionally":[204],"characterized":[205],"series":[207],"N-":[209],"mutant":[213],"transducing":[216],"them":[217],"control":[219],"patient":[221,262],"fibroblasts,":[222],"quantifying":[224],"phenotypic":[226],"effect":[227],"on":[228],"cysteines":[236],"soluble,":[239],"N-terminal":[240],"matrix":[241],"domain":[242],"not":[246],"for":[248],"function.":[250],"Overexpression":[251],"mutants":[255],"only":[256],"affected":[257],"SCO1-2":[261],"fibroblasts.":[263],"To":[264],"further":[265],"characterize":[266],"these":[271,304],"mutants,":[273],"soluble":[274],"each":[277],"variant":[279],"were":[280,301],"expressed":[281],"purified":[283],"bacteria,":[285],"amount":[288],"total":[290],"abundance":[296],"Cu(I)":[298,315],"Cu(II)":[300],"quantified.":[302],"Although":[303],"analyses":[305],"suggested":[306],"one":[308],"mutant,":[309],"C169H,":[311],"binds":[312],"significantly":[313],"more":[314],"than":[316],"wild-type":[318],"protein,":[319],"none":[320],"variants":[324],"exhibited":[325],"furthered":[328],"our":[329],"understanding":[330],"precise":[333],"role":[334],"biogenesis":[339],"site":[343],"II.":[346]},"counts_by_year":[],"updated_date":"2026-09-29T14:09:16.559479","created_date":"2016-06-24T00:00:00"}
