{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,20]],"date-time":"2026-10-20T00:52:20Z","timestamp":1792457540215,"version":"4.5.0"},"reference-count":427,"publisher":"Cambridge University Press (CUP)","license":[{"start":{"date-parts":[[2019,11,25]],"date-time":"2019-11-25T00:00:00Z","timestamp":1574640000000},"content-version":"unspecified","delay-in-days":328,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Quart. Rev. Biophys."],"published-print":{"date-parts":[[2019]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Few proteins have come under such intense scrutiny as superoxide dismutase-1 (SOD1). For almost a century, scientists have dissected its form, function and then later its malfunction in the neurodegenerative disease amyotrophic lateral sclerosis (ALS). We now know SOD1 is a zinc and copper metalloenzyme that clears superoxide as part of our antioxidant defence and respiratory regulation systems. The possibility of reduced structural integrity was suggested by the first crystal structures of human SOD1 even before deleterious mutations in the<jats:italic>sod1<\/jats:italic>gene were linked to the ALS. This concept evolved in the intervening years as an impressive array of biophysical studies examined the characteristics of mutant SOD1 in great detail. We now recognise how ALS-related mutations perturb the SOD1 maturation processes, reduce its ability to fold and reduce its thermal stability and half-life. Mutant SOD1 is therefore predisposed to monomerisation, non-canonical self-interactions, the formation of small misfolded oligomers and ultimately accumulation in the tell-tale insoluble inclusions found within the neurons of ALS patients. We have also seen that several post-translational modifications could push wild-type SOD1 down this toxic pathway. Recently we have come to view ALS as a prion-like disease where both the symptoms, and indeed SOD1 misfolding itself, are transmitted to neighbouring cells. This raises the possibility of intervention after the initial disease presentation. Several small-molecule and biologic-based strategies have been devised which directly target the SOD1 molecule to change the behaviour thought to be responsible for ALS. Here we provide a comprehensive review of the many biophysical advances that sculpted our view of SOD1 biology and the recent work that aims to apply this knowledge for therapeutic outcomes in ALS.<\/jats:p>","DOI":"10.1017\/s003358351900012x","type":"journal-article","created":{"date-parts":[[2019,11,25]],"date-time":"2019-11-25T08:04:24Z","timestamp":1574669064000},"source":"Crossref","is-referenced-by-count":86,"title":["The biophysics of superoxide dismutase-1 and amyotrophic lateral sclerosis"],"prefix":"10.1017","volume":"52","author":[{"given":"Gareth S. A.","family":"Wright","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Svetlana V.","family":"Antonyuk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2854-4718","authenticated-orcid":false,"given":"S. Samar","family":"Hasnain","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"56","published-online":{"date-parts":[[2019,11,25]]},"reference":[{"key":"S003358351900012X_ref426","doi-asserted-by":"publisher","DOI":"10.1007\/s100480050010"},{"key":"S003358351900012X_ref425","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000345363.65799.35"},{"key":"S003358351900012X_ref424","first-page":"449","article-title":"Study of the etiology of amyotrophic lateral sclerosis","volume":"29","author":"Zil'ber","year":"1963","journal-title":"Bulletin of the World Health Organization"},{"key":"S003358351900012X_ref423","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1800187115"},{"key":"S003358351900012X_ref422","first-page":"4817376","article-title":"Production of human Cu,Zn SOD with higher activity and lower toxicity in E. coli via mutation of free cysteine residues","volume":"2017","author":"Zhang","year":"2017","journal-title":"Biomed Research International"},{"key":"S003358351900012X_ref421","doi-asserted-by":"publisher","DOI":"10.1016\/j.freeradbiomed.2004.08.026"},{"key":"S003358351900012X_ref420","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M302051200"},{"key":"S003358351900012X_ref419","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M111.218842"},{"key":"S003358351900012X_ref417","doi-asserted-by":"publisher","DOI":"10.1042\/bj3180889"},{"key":"S003358351900012X_ref415","doi-asserted-by":"publisher","DOI":"10.3109\/17482961003636874"},{"key":"S003358351900012X_ref414","doi-asserted-by":"publisher","DOI":"10.1021\/jacs.8b08141"},{"key":"S003358351900012X_ref409","doi-asserted-by":"publisher","DOI":"10.1038\/ncomms2750"},{"key":"S003358351900012X_ref408","doi-asserted-by":"publisher","DOI":"10.1042\/BJ20110948"},{"key":"S003358351900012X_ref219","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.18-09-03241.1998"},{"key":"S003358351900012X_ref176","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0305143101"},{"key":"S003358351900012X_ref189","doi-asserted-by":"publisher","DOI":"10.1006\/mcpr.1994.1046"},{"key":"S003358351900012X_ref46","doi-asserted-by":"publisher","DOI":"10.1021\/ja211591n"},{"key":"S003358351900012X_ref77","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2012.08.010"},{"key":"S003358351900012X_ref343","doi-asserted-by":"publisher","DOI":"10.7554\/eLife.07296"},{"key":"S003358351900012X_ref175","doi-asserted-by":"publisher","DOI":"10.1016\/S0969-2126(03)00155-2"},{"key":"S003358351900012X_ref139","doi-asserted-by":"crossref","first-page":"1578","DOI":"10.1016\/S0021-9258(18)49375-1","article-title":"Xanthine oxidase: III. Sulfite oxidation as an ultra sensitive assay","volume":"233","author":"Fridovich","year":"1958","journal-title":"Journal of Biological Chemistry"},{"key":"S003358351900012X_ref418","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0700477104"},{"key":"S003358351900012X_ref172","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.71.12.4864"},{"key":"S003358351900012X_ref199","doi-asserted-by":"publisher","DOI":"10.1007\/s004010050983"},{"key":"S003358351900012X_ref416","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.272.14.8861"},{"key":"S003358351900012X_ref171","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1967.00470210008002"},{"key":"S003358351900012X_ref300","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddp260"},{"key":"S003358351900012X_ref192","doi-asserted-by":"publisher","DOI":"10.1097\/01.jnen.0000248545.36046.3c"},{"key":"S003358351900012X_ref168","doi-asserted-by":"crossref","first-page":"7043","DOI":"10.1016\/S0021-9258(19)44691-7","article-title":"Subunit structure of human superoxide dismutase","volume":"247","author":"Hartz","year":"1972","journal-title":"Journal of Biological Chemistry"},{"key":"S003358351900012X_ref190","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awh005"},{"key":"S003358351900012X_ref204","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmb.2008.09.045"},{"key":"S003358351900012X_ref165","doi-asserted-by":"publisher","DOI":"10.1042\/bj3530411"},{"key":"S003358351900012X_ref209","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-010-0646-5"},{"key":"S003358351900012X_ref313","doi-asserted-by":"publisher","DOI":"10.1038\/ng0596-43"},{"key":"S003358351900012X_ref160","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1312245111"},{"key":"S003358351900012X_ref181","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1320786110"},{"key":"S003358351900012X_ref157","doi-asserted-by":"publisher","DOI":"10.1016\/j.freeradbiomed.2015.03.022"},{"key":"S003358351900012X_ref284","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2004.06.021"},{"key":"S003358351900012X_ref156","doi-asserted-by":"publisher","DOI":"10.1021\/jacs.8b13025"},{"key":"S003358351900012X_ref427","doi-asserted-by":"publisher","DOI":"10.1006\/jmbi.2000.4186"},{"key":"S003358351900012X_ref135","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0011552"},{"key":"S003358351900012X_ref155","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1400137111"},{"key":"S003358351900012X_ref152","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2013.04.007"},{"key":"S003358351900012X_ref149","doi-asserted-by":"publisher","DOI":"10.1002\/mus.21750"},{"key":"S003358351900012X_ref148","doi-asserted-by":"publisher","DOI":"10.1016\/j.abb.2009.09.020"},{"key":"S003358351900012X_ref245","doi-asserted-by":"publisher","DOI":"10.1002\/1873-3468.13055"},{"key":"S003358351900012X_ref319","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-2836(76)80050-2"},{"key":"S003358351900012X_ref187","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.220417997"},{"key":"S003358351900012X_ref150","doi-asserted-by":"publisher","DOI":"10.1126\/science.119.3097.623"},{"key":"S003358351900012X_ref196","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0902505106"},{"key":"S003358351900012X_ref70","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.1993.tb07478.x"},{"key":"S003358351900012X_ref137","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbadis.2011.12.001"},{"key":"S003358351900012X_ref185","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/124.7.1461"},{"key":"S003358351900012X_ref134","doi-asserted-by":"crossref","first-page":"2645","DOI":"10.1016\/S0021-9258(19)44055-6","article-title":"On the stability of bovine superoxide dismutase. The effects of metals","volume":"248","author":"Forman","year":"1973","journal-title":"Journal of Biological Chemistry"},{"key":"S003358351900012X_ref197","doi-asserted-by":"publisher","DOI":"10.1097\/00005072-199655100-00009"},{"key":"S003358351900012X_ref162","doi-asserted-by":"crossref","first-page":"1188","DOI":"10.1111\/j.1471-4159.2010.06683.x","article-title":"Intracerebroventricular infusion of monoclonal antibody or its derived Fab fragment against misfolded forms of SOD1 mutant delays mortality in a mouse model of ALS","volume":"113","author":"Gros-Louis","year":"2010","journal-title":"Journal of Neurochemistry"},{"key":"S003358351900012X_ref186","doi-asserted-by":"publisher","DOI":"10.1038\/nature15369"},{"key":"S003358351900012X_ref241","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-017-17815-y"},{"key":"S003358351900012X_ref129","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.2007.04531.x"},{"key":"S003358351900012X_ref170","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M112087200"},{"key":"S003358351900012X_ref288","doi-asserted-by":"publisher","DOI":"10.1016\/0005-2736(76)90272-8"},{"key":"S003358351900012X_ref128","doi-asserted-by":"publisher","DOI":"10.1016\/j.neulet.2004.12.045"},{"key":"S003358351900012X_ref193","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M603337200"},{"key":"S003358351900012X_ref312","doi-asserted-by":"publisher","DOI":"10.1021\/bi030246r"},{"key":"S003358351900012X_ref124","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M300484200"},{"key":"S003358351900012X_ref122","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2018.11.025"},{"key":"S003358351900012X_ref120","doi-asserted-by":"publisher","DOI":"10.1021\/acs.biochem.5b01133"},{"key":"S003358351900012X_ref116","doi-asserted-by":"publisher","DOI":"10.3109\/17482968.2011.551939"},{"key":"S003358351900012X_ref143","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M500482200"},{"key":"S003358351900012X_ref115","doi-asserted-by":"publisher","DOI":"10.1007\/s00018-017-2519-8"},{"key":"S003358351900012X_ref114","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1511308112"},{"key":"S003358351900012X_ref117","doi-asserted-by":"publisher","DOI":"10.1126\/science.8351519"},{"key":"S003358351900012X_ref113","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-017-1688-8"},{"key":"S003358351900012X_ref271","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmb.2014.07.016"},{"key":"S003358351900012X_ref36","doi-asserted-by":"publisher","DOI":"10.1021\/bi9803473"},{"key":"S003358351900012X_ref141","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2015.08.010"},{"key":"S003358351900012X_ref112","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.272.38.23469"},{"key":"S003358351900012X_ref324","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M112088200"},{"key":"S003358351900012X_ref111","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1721022115"},{"key":"S003358351900012X_ref138","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.bi.44.070175.001051"},{"key":"S003358351900012X_ref169","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.35.2.174"},{"key":"S003358351900012X_ref109","doi-asserted-by":"publisher","DOI":"10.1002\/1615-9861(200103)1:3<435::AID-PROT435>3.0.CO;2-Q"},{"key":"S003358351900012X_ref15","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/119.4.1153"},{"key":"S003358351900012X_ref108","doi-asserted-by":"publisher","DOI":"10.1046\/j.1471-4159.1997.69051936.x"},{"key":"S003358351900012X_ref350","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmb.2013.07.018"},{"key":"S003358351900012X_ref178","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/4.3.491"},{"key":"S003358351900012X_ref405","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.R117.789271"},{"key":"S003358351900012X_ref182","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1007\/s004010100399","article-title":"Cuzn superoxide dismutase (SOD1) accumulates in vacuolated mitochondria in transgenic mice expressing amyotrophic lateral sclerosis-linked SOD1 mutations","volume":"102","author":"Jaarsma","year":"2001","journal-title":"Acta Neuropathologica"},{"key":"S003358351900012X_ref103","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M114.586370"},{"key":"S003358351900012X_ref273","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.91.21.9906"},{"key":"S003358351900012X_ref318","doi-asserted-by":"publisher","DOI":"10.1016\/0006-291X(75)90666-X"},{"key":"S003358351900012X_ref232","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbamem.2014.10.002"},{"key":"S003358351900012X_ref102","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1613854114"},{"key":"S003358351900012X_ref107","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0206751"},{"key":"S003358351900012X_ref101","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbrc.2016.10.065"},{"key":"S003358351900012X_ref398","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1098-1004(1997)9:1<69::AID-HUMU14>3.0.CO;2-N"},{"key":"S003358351900012X_ref136","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2018-319386"},{"key":"S003358351900012X_ref97","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2015.11.017"},{"key":"S003358351900012X_ref268","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-015-1431-2"},{"key":"S003358351900012X_ref42","doi-asserted-by":"publisher","DOI":"10.1038\/nature09018"},{"key":"S003358351900012X_ref287","doi-asserted-by":"publisher","DOI":"10.1093\/protein\/12.7.535"},{"key":"S003358351900012X_ref397","doi-asserted-by":"publisher","DOI":"10.15252\/emmm.201606403"},{"key":"S003358351900012X_ref144","doi-asserted-by":"publisher","DOI":"10.1038\/sj.emboj.7600276"},{"key":"S003358351900012X_ref40","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0704307104"},{"key":"S003358351900012X_ref35","doi-asserted-by":"publisher","DOI":"10.1111\/j.1432-1033.1995.855_a.x"},{"key":"S003358351900012X_ref59","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-018-1915-y"},{"key":"S003358351900012X_ref340","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.274.52.36952"},{"key":"S003358351900012X_ref16","doi-asserted-by":"publisher","DOI":"10.1080\/14660820310011700"},{"key":"S003358351900012X_ref3","doi-asserted-by":"publisher","DOI":"10.1021\/acschemneuro.7b00029"},{"key":"S003358351900012X_ref184","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410420518"},{"key":"S003358351900012X_ref307","first-page":"3936","article-title":"Rational discovery of a SOD1 tryptophan oxidation inhibitor with therapeutic potential for amyotrophic lateral sclerosis","volume":"37","author":"Ramu","year":"2018","journal-title":"Journal of Biomolecular Structure and Dynamics"},{"key":"S003358351900012X_ref31","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-015-1514-0"},{"key":"S003358351900012X_ref58","doi-asserted-by":"publisher","DOI":"10.1172\/JCI84360"},{"key":"S003358351900012X_ref364","doi-asserted-by":"publisher","DOI":"10.1002\/jnr.22175"},{"key":"S003358351900012X_ref26","doi-asserted-by":"publisher","DOI":"10.1021\/bi400613h"},{"key":"S003358351900012X_ref277","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijbiomac.2013.07.007"},{"key":"S003358351900012X_ref44","doi-asserted-by":"publisher","DOI":"10.1038\/nchembio.1202"},{"key":"S003358351900012X_ref72","doi-asserted-by":"publisher","DOI":"10.4172\/jpb.1000473"},{"key":"S003358351900012X_ref6","doi-asserted-by":"publisher","DOI":"10.1080\/00207450590914392"},{"key":"S003358351900012X_ref45","doi-asserted-by":"publisher","DOI":"10.1007\/s00775-014-1130-9"},{"key":"S003358351900012X_ref27","doi-asserted-by":"publisher","DOI":"10.1021\/bi4006122"},{"key":"S003358351900012X_ref226","doi-asserted-by":"publisher","DOI":"10.1021\/ja1057136"},{"key":"S003358351900012X_ref191","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awh704"},{"key":"S003358351900012X_ref5","doi-asserted-by":"crossref","first-page":"7339","DOI":"10.1016\/S0021-9258(19)42109-1","article-title":"Bovine erythrocyte superoxide dismutase. Subunit structure and sequence location of the intrasubunit disulfide bond","volume":"249","author":"Abernethy","year":"1974","journal-title":"Journal of Biological Chemistry"},{"key":"S003358351900012X_ref126","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/4.7.1239"},{"key":"S003358351900012X_ref153","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2018.08.021"},{"key":"S003358351900012X_ref100","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbrc.2004.07.010"},{"key":"S003358351900012X_ref174","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmb.2006.09.048"},{"key":"S003358351900012X_ref133","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2009.192310"},{"key":"S003358351900012X_ref283","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.89.13.6109"},{"key":"S003358351900012X_ref346","doi-asserted-by":"publisher","DOI":"10.1016\/j.tibs.2006.12.005"},{"key":"S003358351900012X_ref316","doi-asserted-by":"crossref","first-page":"6368","DOI":"10.1016\/S0021-9258(19)44806-0","article-title":"Two crystal forms of bovine superoxide dismutase","volume":"247","author":"Richardson","year":"1972","journal-title":"Journal of Biological Chemistry"},{"key":"S003358351900012X_ref8","doi-asserted-by":"publisher","DOI":"10.1039\/C4RA03806A"},{"key":"S003358351900012X_ref275","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0601696103"},{"key":"S003358351900012X_ref28","doi-asserted-by":"publisher","DOI":"10.1002\/pmic.201300450"},{"key":"S003358351900012X_ref96","doi-asserted-by":"publisher","DOI":"10.1007\/s11064-011-0679-8"},{"key":"S003358351900012X_ref367","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-2836(03)00355-3"},{"key":"S003358351900012X_ref22","doi-asserted-by":"publisher","DOI":"10.1016\/0304-4165(87)90025-0"},{"key":"S003358351900012X_ref374","doi-asserted-by":"publisher","DOI":"10.1096\/fj.02-0768fje"},{"key":"S003358351900012X_ref320","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.47.5.1336"},{"key":"S003358351900012X_ref13","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/120.10.1723"},{"key":"S003358351900012X_ref146","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M802083200"},{"key":"S003358351900012X_ref50","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2010.03.009"},{"key":"S003358351900012X_ref413","doi-asserted-by":"publisher","DOI":"10.1038\/nn2047"},{"key":"S003358351900012X_ref110","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410410212"},{"key":"S003358351900012X_ref352","doi-asserted-by":"publisher","DOI":"10.1021\/jacs.6b01742"},{"key":"S003358351900012X_ref55","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.2010.06753.x"},{"key":"S003358351900012X_ref123","first-page":"435","article-title":"Untersuchungen \u00fcber die zustandsform des kupfers im blutserum mit hilfe der kataphorese","volume":"286","author":"Eisler","year":"1936","journal-title":"Biochemische Zeitschrift"},{"key":"S003358351900012X_ref281","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-018-31773-z"},{"key":"S003358351900012X_ref360","doi-asserted-by":"publisher","DOI":"10.3389\/fmolb.2016.00055"},{"key":"S003358351900012X_ref158","doi-asserted-by":"publisher","DOI":"10.1016\/j.neulet.2007.09.024"},{"key":"S003358351900012X_ref81","doi-asserted-by":"publisher","DOI":"10.1038\/sj.emboj.7601528"},{"key":"S003358351900012X_ref234","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.262527099"},{"key":"S003358351900012X_ref92","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.273.37.23625"},{"key":"S003358351900012X_ref24","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M406021200"},{"key":"S003358351900012X_ref34","doi-asserted-by":"publisher","DOI":"10.1111\/j.1432-1033.1991.tb15794.x"},{"key":"S003358351900012X_ref195","doi-asserted-by":"publisher","DOI":"10.3389\/fncel.2016.00287"},{"key":"S003358351900012X_ref32","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2011.03.041"},{"key":"S003358351900012X_ref358","doi-asserted-by":"publisher","DOI":"10.1038\/s41419-017-0130-4"},{"key":"S003358351900012X_ref69","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.93.23.12774"},{"key":"S003358351900012X_ref365","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M111.274407"},{"key":"S003358351900012X_ref198","first-page":"611","article-title":"Pathological characterization of astrocytic hyaline inclusions in familial amyotrophic lateral sclerosis","volume":"151","author":"Kato","year":"1997","journal-title":"American Journal of Pathology"},{"key":"S003358351900012X_ref119","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-2836(03)00889-1"},{"key":"S003358351900012X_ref393","doi-asserted-by":"publisher","DOI":"10.1006\/nbdi.2002.0498"},{"key":"S003358351900012X_ref18","doi-asserted-by":"publisher","DOI":"10.1038\/srep41141"},{"key":"S003358351900012X_ref94","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0807058105"},{"key":"S003358351900012X_ref161","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/dds399"},{"key":"S003358351900012X_ref51","doi-asserted-by":"publisher","DOI":"10.1016\/0005-2795(73)90198-0"},{"key":"S003358351900012X_ref202","doi-asserted-by":"publisher","DOI":"10.1007\/s004010000226"},{"key":"S003358351900012X_ref180","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2015.02.034"},{"key":"S003358351900012X_ref14","doi-asserted-by":"publisher","DOI":"10.1002\/ana.10850"},{"key":"S003358351900012X_ref99","doi-asserted-by":"publisher","DOI":"10.1002\/prot.24688"},{"key":"S003358351900012X_ref48","doi-asserted-by":"publisher","DOI":"10.1021\/acschemneuro.8b00491"},{"key":"S003358351900012X_ref183","doi-asserted-by":"publisher","DOI":"10.1021\/bi00552a005"},{"key":"S003358351900012X_ref30","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-014-1342-7"},{"key":"S003358351900012X_ref378","doi-asserted-by":"crossref","first-page":"5677","DOI":"10.1016\/S0021-9258(20)79780-2","article-title":"The crystal structure of bovine Cu2+,Zn2+ superoxide dismutase at 5.5-A resolution","volume":"249","author":"Thomas","year":"1974","journal-title":"Journal of Biological Chemistry"},{"key":"S003358351900012X_ref412","doi-asserted-by":"publisher","DOI":"10.1038\/s41419-017-0106-4"},{"key":"S003358351900012X_ref64","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.91.17.8292"},{"key":"S003358351900012X_ref20","doi-asserted-by":"publisher","DOI":"10.1002\/pro.3094"},{"key":"S003358351900012X_ref335","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pbio.3000141"},{"key":"S003358351900012X_ref71","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.1993.tb07478.x"},{"key":"S003358351900012X_ref66","doi-asserted-by":"publisher","DOI":"10.1038\/nn.2660"},{"key":"S003358351900012X_ref315","doi-asserted-by":"publisher","DOI":"10.1038\/268495a0"},{"key":"S003358351900012X_ref389","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0712209105"},{"key":"S003358351900012X_ref142","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M702941200"},{"key":"S003358351900012X_ref4","doi-asserted-by":"publisher","DOI":"10.1002\/humu.22157"},{"key":"S003358351900012X_ref349","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M707751200"},{"key":"S003358351900012X_ref127","doi-asserted-by":"publisher","DOI":"10.1006\/bbrc.2000.3581"},{"key":"S003358351900012X_ref53","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0607423103"},{"key":"S003358351900012X_ref351","doi-asserted-by":"publisher","DOI":"10.1021\/ja407801x"},{"key":"S003358351900012X_ref39","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M506497200"},{"key":"S003358351900012X_ref87","doi-asserted-by":"publisher","DOI":"10.1038\/s41467-018-04114-x"},{"key":"S003358351900012X_ref37","doi-asserted-by":"publisher","DOI":"10.1046\/j.1432-1033.2002.02840.x"},{"key":"S003358351900012X_ref43","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1207493109"},{"key":"S003358351900012X_ref9","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2011.08.031"},{"key":"S003358351900012X_ref54","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2006.12.015"},{"key":"S003358351900012X_ref382","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-017-1726-6"},{"key":"S003358351900012X_ref257","doi-asserted-by":"publisher","DOI":"10.1016\/0014-5793(76)80550-9"},{"key":"S003358351900012X_ref21","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1038\/ng1293-323","article-title":"Mild ALS in Japan associated with novel SOD mutation","volume":"5","author":"Aoki","year":"1993","journal-title":"Nature Genetics"},{"key":"S003358351900012X_ref56","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1419228112"},{"key":"S003358351900012X_ref285","doi-asserted-by":"publisher","DOI":"10.1021\/ja01360a004"},{"key":"S003358351900012X_ref33","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2016-313521"},{"key":"S003358351900012X_ref292","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0184384"},{"key":"S003358351900012X_ref177","doi-asserted-by":"publisher","DOI":"10.1042\/BSR20120029"},{"key":"S003358351900012X_ref62","doi-asserted-by":"publisher","DOI":"10.1021\/acs.jpcb.8b10774"},{"key":"S003358351900012X_ref327","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/3.6.981"},{"key":"S003358351900012X_ref399","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1205829109"},{"key":"S003358351900012X_ref154","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-019-43164-z"},{"key":"S003358351900012X_ref164","doi-asserted-by":"publisher","DOI":"10.1016\/S0006-291X(05)81443-3"},{"key":"S003358351900012X_ref2","doi-asserted-by":"publisher","DOI":"10.1021\/acschemneuro.6b00048"},{"key":"S003358351900012X_ref75","doi-asserted-by":"publisher","DOI":"10.1021\/acs.biochem.5b01187"},{"key":"S003358351900012X_ref17","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M113.487231"},{"key":"S003358351900012X_ref67","doi-asserted-by":"publisher","DOI":"10.1017\/S0317167100034004"},{"key":"S003358351900012X_ref303","doi-asserted-by":"publisher","DOI":"10.1126\/science.284.5415.805"},{"key":"S003358351900012X_ref90","doi-asserted-by":"crossref","first-page":"723","DOI":"10.1016\/S0021-9258(18)63323-X","article-title":"The presence of zinc in human cytocuprein and some properties of the apoprotein","volume":"245","author":"Carrico","year":"1970","journal-title":"Journal of Biological Chemistry"},{"key":"S003358351900012X_ref23","doi-asserted-by":"crossref","first-page":"16969","DOI":"10.1016\/S0021-9258(18)45479-8","article-title":"Glycation and inactivation of human Cu-Zn-superoxide dismutase. Identification of the in vitro glycated sites","volume":"262","author":"Arai","year":"1987","journal-title":"Journal of Biological Chemistry"},{"key":"S003358351900012X_ref93","doi-asserted-by":"publisher","DOI":"10.3390\/antiox6040082"},{"key":"S003358351900012X_ref342","doi-asserted-by":"publisher","DOI":"10.1080\/146608202760196039"},{"key":"S003358351900012X_ref379","doi-asserted-by":"publisher","DOI":"10.1016\/0022-2836(81)90515-5"},{"key":"S003358351900012X_ref63","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbabio.2012.12.002"},{"key":"S003358351900012X_ref188","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/3.4.649"},{"key":"S003358351900012X_ref332","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuroscience.2015.09.027"},{"key":"S003358351900012X_ref73","first-page":"674","article-title":"Achromatic regions of tetrazolium stained starch gels: inherited electrophoretic variation","volume":"19","author":"Brewer","year":"1967","journal-title":"American Journal of Human Genetics"},{"key":"S003358351900012X_ref132","doi-asserted-by":"publisher","DOI":"10.1016\/j.str.2016.08.011"},{"key":"S003358351900012X_ref74","doi-asserted-by":"publisher","DOI":"10.1002\/pro.2803"},{"key":"S003358351900012X_ref159","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1102645108"},{"key":"S003358351900012X_ref78","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0401175101"},{"key":"S003358351900012X_ref262","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2010.077180"},{"key":"S003358351900012X_ref76","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1115009109"},{"key":"S003358351900012X_ref52","doi-asserted-by":"publisher","DOI":"10.1111\/j.1601-5223.1973.tb01093.x"},{"key":"S003358351900012X_ref145","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0602048103"},{"key":"S003358351900012X_ref105","doi-asserted-by":"publisher","DOI":"10.1089\/ars.2009.2545"},{"key":"S003358351900012X_ref247","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1098\/rspb.1938.0058","article-title":"Haemocuprein and hepatocuprein, copper-protein compounds of blood and liver in mammals","volume":"126","author":"Mann","year":"1938","journal-title":"Proceedings of the Royal Society of London B Biological Sciences"},{"key":"S003358351900012X_ref194","doi-asserted-by":"publisher","DOI":"10.1038\/nature07195"},{"key":"S003358351900012X_ref79","doi-asserted-by":"publisher","DOI":"10.1126\/science.281.5384.1851"},{"key":"S003358351900012X_ref401","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M809687200"},{"key":"S003358351900012X_ref173","doi-asserted-by":"crossref","first-page":"683","DOI":"10.1016\/S0021-9258(18)56885-X","article-title":"The reduction of cytochrome c by milk xanthine oxidase","volume":"178","author":"Horecker","year":"1949","journal-title":"Journal of Biological Chemistry"},{"key":"S003358351900012X_ref80","doi-asserted-by":"publisher","DOI":"10.1016\/S0896-6273(00)80272-X"},{"key":"S003358351900012X_ref151","doi-asserted-by":"publisher","DOI":"10.1038\/306287a0"},{"key":"S003358351900012X_ref373","doi-asserted-by":"publisher","DOI":"10.1038\/306284a0"},{"key":"S003358351900012X_ref131","doi-asserted-by":"publisher","DOI":"10.1021\/acschemneuro.8b00527"},{"key":"S003358351900012X_ref334","doi-asserted-by":"publisher","DOI":"10.1111\/j.1468-1331.1995.tb00141.x"},{"key":"S003358351900012X_ref354","doi-asserted-by":"publisher","DOI":"10.1016\/0304-3940(94)90956-3"},{"key":"S003358351900012X_ref68","doi-asserted-by":"publisher","DOI":"10.3389\/fnagi.2014.00110"},{"key":"S003358351900012X_ref10","doi-asserted-by":"publisher","DOI":"10.1021\/bi201370r"},{"key":"S003358351900012X_ref299","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddn063"},{"key":"S003358351900012X_ref12","doi-asserted-by":"publisher","DOI":"10.1038\/ng0595-61"},{"key":"S003358351900012X_ref89","doi-asserted-by":"crossref","first-page":"6087","DOI":"10.1016\/S0021-9258(18)63509-4","article-title":"Isolation of human hepatocuprein and cerebrocuprein their identity with erythrocuprein","volume":"244","author":"Carrico","year":"1969","journal-title":"Journal of Biological Chemistry"},{"key":"S003358351900012X_ref60","doi-asserted-by":"publisher","DOI":"10.18388\/abp.2014_1906"},{"key":"S003358351900012X_ref291","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0035050"},{"key":"S003358351900012X_ref385","doi-asserted-by":"crossref","first-page":"2405","DOI":"10.1016\/S0021-9258(17)41960-0","article-title":"Identification of oxidized histidine generated at the active site of Cu,Zn-superoxide dismutase exposed to H2O2. Selective generation of 2-oxo-histidine at the histidine 118","volume":"269","author":"Uchida","year":"1994","journal-title":"Journal of Biological Chemistry"},{"key":"S003358351900012X_ref121","doi-asserted-by":"publisher","DOI":"10.1097\/00005072-199705000-00008"},{"key":"S003358351900012X_ref326","doi-asserted-by":"publisher","DOI":"10.1038\/362059a0"},{"key":"S003358351900012X_ref200","doi-asserted-by":"publisher","DOI":"10.1016\/S0304-3940(01)02212-1"},{"key":"S003358351900012X_ref201","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1007\/s004010000355","article-title":"Copper chaperone for superoxide dismutase co-aggregates with superoxide dismutase 1 (SOD1) in neuronal Lewy body-like hyaline inclusions: an immunohistochemical study on familial amyotrophic lateral sclerosis with SOD1 gene mutation","volume":"102","author":"Kato","year":"2001","journal-title":"Acta Neuropathologica"},{"key":"S003358351900012X_ref203","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddn226"},{"key":"S003358351900012X_ref297","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1308531111"},{"key":"S003358351900012X_ref47","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M117.789610"},{"key":"S003358351900012X_ref205","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmb.2010.02.034"},{"key":"S003358351900012X_ref402","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2016.01.020"},{"key":"S003358351900012X_ref206","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmb.2012.09.009"},{"key":"S003358351900012X_ref411","doi-asserted-by":"publisher","DOI":"10.1038\/gim.2012.50"},{"key":"S003358351900012X_ref207","doi-asserted-by":"crossref","first-page":"6176","DOI":"10.1016\/S0021-9258(18)62675-4","article-title":"Superoxide dismutase from Escherichia coli B. A new manganese-containing enzyme","volume":"245","author":"Keele","year":"1970","journal-title":"Journal of Biological Chemistry"},{"key":"S003358351900012X_ref251","doi-asserted-by":"publisher","DOI":"10.15252\/embj.201695957"},{"key":"S003358351900012X_ref210","first-page":"569","article-title":"X-ray crystallography and computational docking for the detection and development of protein-ligand interactions","volume":"20","author":"Kershaw","year":"2013","journal-title":"Current Medicinal Chemistry"},{"key":"S003358351900012X_ref211","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0150133"},{"key":"S003358351900012X_ref212","first-page":"1","article-title":"Comprehensive analysis to explain reduced or increased SOD1 enzymatic activity in ALS patients and their relatives","volume":"0","author":"Keskin","year":"2017","journal-title":"Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration"},{"key":"S003358351900012X_ref213","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0406650101"},{"key":"S003358351900012X_ref260","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.68.5.1024"},{"key":"S003358351900012X_ref214","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M502230200"},{"key":"S003358351900012X_ref140","doi-asserted-by":"crossref","first-page":"1836","DOI":"10.1016\/S0021-9258(19)63312-0","article-title":"Detection of free radicals generated during enzymic oxidations by the initiation of sulfite oxidation","volume":"236","author":"Fridovich","year":"1961","journal-title":"Journal of Biological Chemistry"},{"key":"S003358351900012X_ref376","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1128\/IAI.16.1.20-25.1977","article-title":"Superoxide dismutase in anaerobic bacteria of clinical significance","volume":"16","author":"Tally","year":"1977","journal-title":"Infection and Immunity"},{"key":"S003358351900012X_ref216","doi-asserted-by":"crossref","first-page":"4839","DOI":"10.1016\/S0021-9258(19)44987-9","article-title":"A direct demonstration of the catalytic action of superoxide dismutase through the use of pulse radiolysis","volume":"247","author":"Klug","year":"1972","journal-title":"Journal of Biological Chemistry"},{"key":"S003358351900012X_ref38","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M506637200"},{"key":"S003358351900012X_ref272","doi-asserted-by":"publisher","DOI":"10.1063\/1.1749353"},{"key":"S003358351900012X_ref396","doi-asserted-by":"publisher","DOI":"10.1006\/nbdi.2001.0443"},{"key":"S003358351900012X_ref217","doi-asserted-by":"publisher","DOI":"10.3109\/17482968.2012.686512"},{"key":"S003358351900012X_ref29","doi-asserted-by":"publisher","DOI":"10.1111\/jnc.12451"},{"key":"S003358351900012X_ref95","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M115.666503"},{"key":"S003358351900012X_ref218","doi-asserted-by":"publisher","DOI":"10.1016\/S0304-3940(98)00800-3"},{"key":"S003358351900012X_ref384","doi-asserted-by":"publisher","DOI":"10.1038\/ncomms4446"},{"key":"S003358351900012X_ref391","doi-asserted-by":"publisher","DOI":"10.1016\/j.ibror.2017.03.001"},{"key":"S003358351900012X_ref7","doi-asserted-by":"publisher","DOI":"10.1002\/ana.10369"},{"key":"S003358351900012X_ref220","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/3.12.2261"},{"key":"S003358351900012X_ref222","doi-asserted-by":"crossref","first-page":"724","DOI":"10.1038\/11489","article-title":"Crystal structure of the copper chaperone for superoxide dismutase","volume":"6","author":"Lamb","year":"1999","journal-title":"Natural Structural Biology"},{"key":"S003358351900012X_ref223","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1503328112"},{"key":"S003358351900012X_ref224","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M113.470740"},{"key":"S003358351900012X_ref244","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.1233-11.2012"},{"key":"S003358351900012X_ref84","doi-asserted-by":"publisher","DOI":"10.1111\/j.1432-1033.1975.tb02234.x"},{"key":"S003358351900012X_ref383","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.91.14.6384"},{"key":"S003358351900012X_ref225","doi-asserted-by":"publisher","DOI":"10.1159\/000437208"},{"key":"S003358351900012X_ref106","doi-asserted-by":"publisher","DOI":"10.1172\/JCI80705"},{"key":"S003358351900012X_ref328","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.91.10.4155"},{"key":"S003358351900012X_ref227","doi-asserted-by":"publisher","DOI":"10.1038\/nature19057"},{"key":"S003358351900012X_ref83","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M109.086074"},{"key":"S003358351900012X_ref228","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M110.186999"},{"key":"S003358351900012X_ref264","doi-asserted-by":"publisher","DOI":"10.1046\/j.1468-1331.1998.540389.x"},{"key":"S003358351900012X_ref229","doi-asserted-by":"publisher","DOI":"10.1016\/0005-2736(81)90007-9"},{"key":"S003358351900012X_ref230","doi-asserted-by":"crossref","first-page":"21612","DOI":"10.1016\/S0021-9258(18)45784-5","article-title":"Contribution of conformational stability and reversibility of unfolding to the increased thermostability of human and bovine superoxide dismutase mutated at free cysteines","volume":"265","author":"Lepock","year":"1990","journal-title":"Journal of Biological Chemistry"},{"key":"S003358351900012X_ref221","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.5.3.182"},{"key":"S003358351900012X_ref231","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2012.02.018"},{"key":"S003358351900012X_ref11","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M115.708230"},{"key":"S003358351900012X_ref233","doi-asserted-by":"crossref","first-page":"20578","DOI":"10.18632\/oncotarget.3987","article-title":"Acetylation at lysine 71 inactivates superoxide dismutase 1 and sensitizes cancer cells to genotoxic agents","volume":"6","author":"Lin","year":"2015","journal-title":"Oncotarget"},{"key":"S003358351900012X_ref235","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0403979101"},{"key":"S003358351900012X_ref410","doi-asserted-by":"publisher","DOI":"10.1038\/srep27691"},{"key":"S003358351900012X_ref208","doi-asserted-by":"crossref","first-page":"2875","DOI":"10.1016\/S0021-9258(18)62263-X","article-title":"Further characterization of bovine superoxide dismutase and its isolation from bovine heart","volume":"246","author":"Keele","year":"1971","journal-title":"Journal of Biological Chemistry"},{"key":"S003358351900012X_ref236","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0501957102"},{"key":"S003358351900012X_ref355","doi-asserted-by":"publisher","DOI":"10.1097\/00005072-199604000-00011"},{"key":"S003358351900012X_ref91","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0308298101"},{"key":"S003358351900012X_ref238","doi-asserted-by":"publisher","DOI":"10.1172\/JCI100432"},{"key":"S003358351900012X_ref239","doi-asserted-by":"publisher","DOI":"10.3109\/17482960902898069"},{"key":"S003358351900012X_ref179","doi-asserted-by":"publisher","DOI":"10.3109\/21678421.2013.822515"},{"key":"S003358351900012X_ref215","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M113.542613"},{"key":"S003358351900012X_ref240","doi-asserted-by":"publisher","DOI":"10.1038\/ncomms6502"},{"key":"S003358351900012X_ref246","doi-asserted-by":"publisher","DOI":"10.1038\/142148a0"},{"key":"S003358351900012X_ref242","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.93.22.12240"},{"key":"S003358351900012X_ref243","article-title":"The contrasting effect of macromolecular crowding on amyloid fibril formation","volume":"7","author":"Ma","year":"2012","journal-title":"PLoS ONE"},{"key":"S003358351900012X_ref353","doi-asserted-by":"publisher","DOI":"10.1267\/ahc.26.619"},{"key":"S003358351900012X_ref248","doi-asserted-by":"publisher","DOI":"10.1046\/j.1471-4159.1997.69020675.x"},{"key":"S003358351900012X_ref19","doi-asserted-by":"publisher","DOI":"10.1021\/jm9017948"},{"key":"S003358351900012X_ref250","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2007.06.003"},{"key":"S003358351900012X_ref252","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200504050"},{"key":"S003358351900012X_ref88","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-2836(02)01090-2"},{"key":"S003358351900012X_ref253","doi-asserted-by":"publisher","DOI":"10.1002\/ana.10605"},{"key":"S003358351900012X_ref255","doi-asserted-by":"publisher","DOI":"10.3389\/fnins.2016.00499"},{"key":"S003358351900012X_ref279","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0005004"},{"key":"S003358351900012X_ref256","doi-asserted-by":"publisher","DOI":"10.3109\/21678421.2013.824000"},{"key":"S003358351900012X_ref344","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1611418113"},{"key":"S003358351900012X_ref258","doi-asserted-by":"crossref","first-page":"5753","DOI":"10.1016\/S0021-9258(18)91929-0","article-title":"The reduction of cytochrome c by milk xanthine oxidase","volume":"243","author":"McCord","year":"1968","journal-title":"Journal of Biological Chemistry"},{"key":"S003358351900012X_ref118","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0602046103"},{"key":"S003358351900012X_ref259","doi-asserted-by":"crossref","first-page":"6049","DOI":"10.1016\/S0021-9258(18)63504-5","article-title":"Superoxide dismutase. An enzymic function for erythrocuprein (hemocuprein)","volume":"244","author":"McCord","year":"1969","journal-title":"Journal of Biological Chemistry"},{"key":"S003358351900012X_ref263","doi-asserted-by":"publisher","DOI":"10.1085\/jgp.37.4.433"},{"key":"S003358351900012X_ref323","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.98.3.944"},{"key":"S003358351900012X_ref265","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1621227114"},{"key":"S003358351900012X_ref266","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmb.2010.04.019"},{"key":"S003358351900012X_ref267","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1017275108"},{"key":"S003358351900012X_ref269","doi-asserted-by":"publisher","DOI":"10.1016\/0304-3940(96)12701-4"},{"key":"S003358351900012X_ref270","doi-asserted-by":"publisher","DOI":"10.1002\/ncn3.8"},{"key":"S003358351900012X_ref274","doi-asserted-by":"publisher","DOI":"10.1007\/BF00571500"},{"key":"S003358351900012X_ref41","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0809845106"},{"key":"S003358351900012X_ref276","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0812046106"},{"key":"S003358351900012X_ref278","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-510X(99)00216-6"},{"key":"S003358351900012X_ref85","doi-asserted-by":"publisher","DOI":"10.3109\/21678421.2014.966312"},{"key":"S003358351900012X_ref280","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.92.4.954"},{"key":"S003358351900012X_ref329","doi-asserted-by":"publisher","DOI":"10.1046\/j.1471-4159.1999.0720422.x"},{"key":"S003358351900012X_ref282","doi-asserted-by":"crossref","first-page":"16215","DOI":"10.1016\/S0021-9258(18)66701-8","article-title":"Crystallographic characterization of recombinant human CuZn superoxide dismutase","volume":"261","author":"Parge","year":"1986","journal-title":"Journal of Biological Chemistry"},{"key":"S003358351900012X_ref167","doi-asserted-by":"crossref","first-page":"545","DOI":"10.1002\/pro.5560070302","article-title":"Subunit asymmetry in the three-dimensional structure of a human CuZnSOD mutant found in familial amyotrophic lateral sclerosis","volume":"7","author":"Hart","year":"1998","journal-title":"Protein Science Publication Protein Society"},{"key":"S003358351900012X_ref286","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbapap.2009.11.004"},{"key":"S003358351900012X_ref289","first-page":"190a","article-title":"Distribution of superoxide dismutase in the erythrocyte membrane","volume":"25","author":"Petkau","year":"1979","journal-title":"Biophysical Journal"},{"key":"S003358351900012X_ref290","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddt249"},{"key":"S003358351900012X_ref125","doi-asserted-by":"publisher","DOI":"10.1038\/nsb935"},{"key":"S003358351900012X_ref61","doi-asserted-by":"publisher","DOI":"10.1021\/jp404500b"},{"key":"S003358351900012X_ref293","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-018-32835-y"},{"key":"S003358351900012X_ref294","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.1957.tb12081.x"},{"key":"S003358351900012X_ref261","doi-asserted-by":"publisher","DOI":"10.1016\/j.freeradbiomed.2010.06.018"},{"key":"S003358351900012X_ref65","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.270.7.3234"},{"key":"S003358351900012X_ref295","doi-asserted-by":"publisher","DOI":"10.1021\/ja066690+"},{"key":"S003358351900012X_ref296","first-page":"592","article-title":"Identification of new mutations in the Cu\/Zn superoxide dismutase gene of patients with familial amyotrophic lateral sclerosis","volume":"56","author":"Pramatarova","year":"1995","journal-title":"American Journal of Human Genetics"},{"key":"S003358351900012X_ref298","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1516725113"},{"key":"S003358351900012X_ref301","doi-asserted-by":"publisher","DOI":"10.2307\/3571096"},{"key":"S003358351900012X_ref302","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.92.7.3024"},{"key":"S003358351900012X_ref305","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M313295200"},{"key":"S003358351900012X_ref322","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.RA118.007095"},{"key":"S003358351900012X_ref306","doi-asserted-by":"publisher","DOI":"10.1038\/nm1559"},{"key":"S003358351900012X_ref249","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1016\/S0021-9258(18)70331-1","article-title":"Studies on copper metabolism XXVII. The isolation and properties of an erythrocyte cuproprotein (Erythrocuprein)","volume":"234","author":"Markowitz","year":"1959","journal-title":"Journal of Biological Chemistry"},{"key":"S003358351900012X_ref309","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M117.805283"},{"key":"S003358351900012X_ref310","doi-asserted-by":"publisher","DOI":"10.1021\/acschemneuro.8b00086"},{"key":"S003358351900012X_ref311","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e3181b6bbbd"},{"key":"S003358351900012X_ref25","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1015463107"},{"key":"S003358351900012X_ref368","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmb.2005.11.081"},{"key":"S003358351900012X_ref314","doi-asserted-by":"publisher","DOI":"10.1021\/bi200614y"},{"key":"S003358351900012X_ref82","first-page":"1292","article-title":"On intracellular inclusions in familial amyotrophic lateral sclerosis","volume":"3","author":"Bunina","year":"1962","journal-title":"S.S. Korsakov Neuropathology and Psychiatry Magazine"},{"key":"S003358351900012X_ref49","doi-asserted-by":"publisher","DOI":"10.1016\/0014-5793(80)81044-1"},{"key":"S003358351900012X_ref347","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M600623200"},{"key":"S003358351900012X_ref317","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.72.4.1349"},{"key":"S003358351900012X_ref321","unstructured":"Robberecht W , Rousseau F and Schymkowitz J (2016) Single domain antibodies against sod1 and their use in medicine. Patents US20160115245A1 and WO2014191493A1."},{"key":"S003358351900012X_ref130","doi-asserted-by":"publisher","DOI":"10.1242\/jcs.209122"},{"key":"S003358351900012X_ref147","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M110.113597"},{"key":"S003358351900012X_ref325","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0502515102"},{"key":"S003358351900012X_ref371","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2010.09.007"},{"key":"S003358351900012X_ref308","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0210752"},{"key":"S003358351900012X_ref330","doi-asserted-by":"crossref","first-page":"3157","DOI":"10.1016\/S0021-9258(19)42651-3","article-title":"Electron paramagnetic resonance studies of cobalt-copper bovine superoxide dismutase","volume":"249","author":"Rotilio","year":"1974","journal-title":"Journal of Biological Chemistry"},{"key":"S003358351900012X_ref254","doi-asserted-by":"publisher","DOI":"10.1038\/srep03275"},{"key":"S003358351900012X_ref331","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmb.2005.10.042"},{"key":"S003358351900012X_ref57","doi-asserted-by":"publisher","DOI":"10.1016\/j.neurobiolaging.2013.10.093"},{"key":"S003358351900012X_ref333","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awt097"},{"key":"S003358351900012X_ref336","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijbiomac.2015.01.022"},{"key":"S003358351900012X_ref380","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M210419200"},{"key":"S003358351900012X_ref104","doi-asserted-by":"publisher","DOI":"10.3109\/17482968.2010.545951"},{"key":"S003358351900012X_ref363","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2009.01.005"},{"key":"S003358351900012X_ref337","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M700765200"},{"key":"S003358351900012X_ref338","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1705091114"},{"key":"S003358351900012X_ref341","doi-asserted-by":"publisher","DOI":"10.1021\/bi100314n"},{"key":"S003358351900012X_ref345","doi-asserted-by":"publisher","DOI":"10.1107\/S139900471500629X"},{"key":"S003358351900012X_ref362","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0610923104"},{"key":"S003358351900012X_ref356","doi-asserted-by":"publisher","DOI":"10.1159\/000111445"},{"key":"S003358351900012X_ref390","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0913021108"},{"key":"S003358351900012X_ref357","doi-asserted-by":"publisher","DOI":"10.1007\/s004010050980"},{"key":"S003358351900012X_ref359","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199105163242001"},{"key":"S003358351900012X_ref361","doi-asserted-by":"publisher","DOI":"10.1007\/BF00182394"},{"key":"S003358351900012X_ref366","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1237797100"},{"key":"S003358351900012X_ref369","doi-asserted-by":"publisher","DOI":"10.1038\/nn823"},{"key":"S003358351900012X_ref370","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(94)92913-0"},{"key":"S003358351900012X_ref339","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000188760.53922.05"},{"key":"S003358351900012X_ref372","doi-asserted-by":"publisher","DOI":"10.1016\/0022-2836(82)90174-7"},{"key":"S003358351900012X_ref375","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.49.2720"},{"key":"S003358351900012X_ref166","doi-asserted-by":"publisher","DOI":"10.1002\/1531-8249(20010201)49:2<267::AID-ANA51>3.0.CO;2-D"},{"key":"S003358351900012X_ref377","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M610119200"},{"key":"S003358351900012X_ref163","doi-asserted-by":"publisher","DOI":"10.1126\/science.8209258"},{"key":"S003358351900012X_ref387","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0606201104"},{"key":"S003358351900012X_ref86","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M801522200"},{"key":"S003358351900012X_ref381","doi-asserted-by":"publisher","DOI":"10.1159\/000089616"},{"key":"S003358351900012X_ref386","doi-asserted-by":"publisher","DOI":"10.1038\/nn1603"},{"key":"S003358351900012X_ref304","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M207356200"},{"key":"S003358351900012X_ref388","doi-asserted-by":"publisher","DOI":"10.3109\/17482968.2011.585163"},{"key":"S003358351900012X_ref348","doi-asserted-by":"publisher","DOI":"10.1016\/S1876-1623(10)79004-0"},{"key":"S003358351900012X_ref392","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.4385-04.2005"},{"key":"S003358351900012X_ref98","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0010627"},{"key":"S003358351900012X_ref394","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pbio.0060170"},{"key":"S003358351900012X_ref1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bpj.2015.01.014"},{"key":"S003358351900012X_ref400","doi-asserted-by":"publisher","DOI":"10.1080\/14660820500467009"},{"key":"S003358351900012X_ref403","doi-asserted-by":"publisher","DOI":"10.1021\/bi8021735"},{"key":"S003358351900012X_ref404","doi-asserted-by":"publisher","DOI":"10.1016\/0896-6273(95)90259-7"},{"key":"S003358351900012X_ref406","doi-asserted-by":"publisher","DOI":"10.1111\/j.1432-1033.1971.tb01229.x"},{"key":"S003358351900012X_ref237","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.5053-11.2012"},{"key":"S003358351900012X_ref395","first-page":"143","article-title":"\u00dcber locker gebundenes kupfer und eisen in blutserum","volume":"190","author":"Warburg","year":"1927","journal-title":"Biochem Z"},{"key":"S003358351900012X_ref407","doi-asserted-by":"publisher","DOI":"10.1093\/protein\/4.3.283"}],"container-title":["Quarterly Reviews of Biophysics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.cambridge.org\/core\/services\/aop-cambridge-core\/content\/view\/S003358351900012X","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,7,27]],"date-time":"2024-07-27T17:25:54Z","timestamp":1722101154000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.cambridge.org\/core\/product\/identifier\/S003358351900012X\/type\/journal_article"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019]]},"references-count":427,"alternative-id":["S003358351900012X"],"URL":"https:\/\/doi.org\/10.1017\/s003358351900012x","relation":{},"ISSN":["0033-5835","1469-8994"],"issn-type":[{"value":"0033-5835","type":"print"},{"value":"1469-8994","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019]]},"article-number":"e12"}}