{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,22]],"date-time":"2026-07-22T03:00:56Z","timestamp":1784689256465,"version":"3.55.0"},"reference-count":118,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2023,10,6]],"date-time":"2023-10-06T00:00:00Z","timestamp":1696550400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Journal of Neurochemistry"],"published-print":{"date-parts":[[2023,11]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Since the first description of Parkinson's disease (PD) over two centuries ago, the recognition of rare types of atypical parkinsonism has introduced a spectrum of related PD\u2010like diseases. Among these is progressive supranuclear palsy (PSP), a neurodegenerative condition that clinically differentiates through the presence of additional symptoms uncommon in PD. As with PD, the initial symptoms of PSP generally present in the sixth decade of life when the underpinning neurodegeneration is already significantly advanced. The causal trigger of neuronal cell loss in PSP is unknown and treatment options are consequently limited. However, converging lines of evidence from the distinct neurodegenerative conditions of PD and amyotrophic lateral sclerosis (ALS) are beginning to provide insights into potential commonalities in PSP pathology and opportunity for novel therapeutic intervention. These include accumulation of the high abundance cuproenzyme superoxide dismutase 1 (SOD1) in an aberrant copper\u2010deficient state, associated evidence for altered availability of the essential micronutrient copper, and evidence for neuroprotection using compounds that can deliver available copper to the central nervous system. Herein, we discuss the existing evidence for SOD1 pathology and copper imbalance in PSP and speculate that treatments able to provide neuroprotection through manipulation of copper availability could be applicable to the treatment of PSP.\n                    <jats:boxed-text content-type=\"graphic\" position=\"anchor\">\n                      <jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" mimetype=\"image\/png\" position=\"anchor\" specific-use=\"enlarged-web-image\" xlink:href=\"graphic\/jnc15978-fig-0003-m.png\">\n                        <jats:alt-text>image<\/jats:alt-text>\n                      <\/jats:graphic>\n                    <\/jats:boxed-text>\n                  <\/jats:p>","DOI":"10.1111\/jnc.15978","type":"journal-article","created":{"date-parts":[[2023,10,6]],"date-time":"2023-10-06T14:53:39Z","timestamp":1696604019000},"page":"337-346","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Fundamental Neurochemistry Review: Copper availability as a potential therapeutic target in progressive supranuclear palsy: Insight from other neurodegenerative diseases"],"prefix":"10.1111","volume":"167","author":[{"ORCID":"https:\/\/orcid.org\/0009-0006-4317-1397","authenticated-orcid":false,"given":"Jessica L.","family":"Billings","sequence":"first","affiliation":[{"name":"Department of Anatomy and Physiology, Faculty of Medicine, Dentistry and Health Sciences The University of Melbourne  Parkville Victoria Australia"},{"name":"The Florey Institute of Neuroscience and Mental Health The University of Melbourne  Parkville Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2657-3566","authenticated-orcid":false,"given":"James B. W.","family":"Hilton","sequence":"additional","affiliation":[{"name":"Department of Anatomy and Physiology, Faculty of Medicine, Dentistry and Health Sciences The University of Melbourne  Parkville Victoria Australia"},{"name":"Centre for Motor Neuron Disease Research, Macquarie Medical School, Faculty of Medicine, Health, and Human Sciences Macquarie University  North Ryde New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0798-5338","authenticated-orcid":false,"given":"Jeffrey R.","family":"Liddell","sequence":"additional","affiliation":[{"name":"Department of Anatomy and Physiology, Faculty of Medicine, Dentistry and Health Sciences The University of Melbourne  Parkville Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5922-7643","authenticated-orcid":false,"given":"Dominic J.","family":"Hare","sequence":"additional","affiliation":[{"name":"School of Mathematical and Physical Sciences University of Technology Sydney, Broadway  Ultimo New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7777-4747","authenticated-orcid":false,"given":"Peter J.","family":"Crouch","sequence":"additional","affiliation":[{"name":"Department of Anatomy and Physiology, Faculty of Medicine, Dentistry and Health Sciences The University of Melbourne  Parkville Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,10,6]]},"reference":[{"key":"e_1_2_13_2_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1432-1327.1998.2570622.x"},{"key":"e_1_2_13_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-019-56930-w"},{"key":"e_1_2_13_4_1","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2020.00180"},{"key":"e_1_2_13_5_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M406021200"},{"key":"e_1_2_13_6_1","doi-asserted-by":"publisher","DOI":"10.3389\/fnagi.2019.00351"},{"key":"e_1_2_13_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.23817"},{"key":"e_1_2_13_8_1","doi-asserted-by":"publisher","DOI":"10.1021\/acs.inorgchem.9b02266"},{"key":"e_1_2_13_9_1","doi-asserted-by":"publisher","DOI":"10.1021\/bi000067z"},{"key":"e_1_2_13_10_1","doi-asserted-by":"publisher","DOI":"10.1039\/C4CS00138A"},{"key":"e_1_2_13_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/acel.13225"},{"key":"e_1_2_13_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0076-6879(02)49320-2"},{"key":"e_1_2_13_13_1","first-page":"775","article-title":"Uptake and toxicity of copper oxide nanoparticles in cultured primary brain astrocytes","volume":"8","author":"Bulcke F.","year":"2014","journal-title":"Nanotoxicology"},{"key":"e_1_2_13_14_1","doi-asserted-by":"publisher","DOI":"10.1177\/1073858414561795"},{"key":"e_1_2_13_15_1","doi-asserted-by":"publisher","DOI":"10.1110\/ps.8.5.985"},{"key":"e_1_2_13_16_1","doi-asserted-by":"publisher","DOI":"10.15252\/embj.201695810"},{"key":"e_1_2_13_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0006-8993(02)02244-8"},{"key":"e_1_2_13_18_1","doi-asserted-by":"publisher","DOI":"10.1080\/13543784.2022.2087179"},{"key":"e_1_2_13_19_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0809057106"},{"key":"e_1_2_13_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.2011.07402.x"},{"key":"e_1_2_13_21_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.272.38.23469"},{"key":"e_1_2_13_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neurobiolaging.2013.09.034"},{"key":"e_1_2_13_23_1","doi-asserted-by":"publisher","DOI":"10.1039\/C2MT20151H"},{"key":"e_1_2_13_24_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/114.4.1953"},{"key":"e_1_2_13_25_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1116227108"},{"key":"e_1_2_13_26_1","doi-asserted-by":"publisher","DOI":"10.3389\/fnagi.2013.00009"},{"key":"e_1_2_13_27_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.1644-07.2007"},{"key":"e_1_2_13_28_1","doi-asserted-by":"publisher","DOI":"10.3233\/JAD-2006-102-305"},{"key":"e_1_2_13_29_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-002-0587-8"},{"key":"e_1_2_13_30_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-011-0805-3"},{"key":"e_1_2_13_31_1","doi-asserted-by":"publisher","DOI":"10.1002\/9780470122860.ch2"},{"key":"e_1_2_13_32_1","doi-asserted-by":"publisher","DOI":"10.3389\/fnmol.2015.00022"},{"key":"e_1_2_13_33_1","doi-asserted-by":"publisher","DOI":"10.3389\/fncel.2013.00240"},{"key":"e_1_2_13_34_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M802083200"},{"key":"e_1_2_13_35_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.emboj.7600276"},{"key":"e_1_2_13_36_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bjp.0706416"},{"key":"e_1_2_13_37_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI71714"},{"key":"e_1_2_13_38_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.38.7.1031"},{"key":"e_1_2_13_39_1","doi-asserted-by":"publisher","DOI":"10.1021\/bi982199m"},{"key":"e_1_2_13_40_1","doi-asserted-by":"publisher","DOI":"10.1039\/C0DT01176B"},{"key":"e_1_2_13_41_1","doi-asserted-by":"crossref","unstructured":"Hilton J. B. Kysenius K. Liddell J. R. Rautengarten C. Mercer S. W. Paul B. Beckman J. McLean C. White A. Donnelly P. Bush A. Hare D. Roberts B. &Crouch P. J.(2020).Disrupted copper availability in sporadic ALS: Implications for CuII(atsm) as a treatment option. bioRxiv.","DOI":"10.1101\/2020.04.17.047704"},{"key":"e_1_2_13_42_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.expneurol.2018.06.006"},{"key":"e_1_2_13_43_1","doi-asserted-by":"publisher","DOI":"10.1038\/srep42292"},{"key":"e_1_2_13_44_1","first-page":"193","article-title":"Assessing tumor hypoxia by positron emission tomography with Cu\u2010ATSM","volume":"53","author":"Holland J. P.","year":"2009","journal-title":"The Quarterly Journal of Nuclear Medicine and Molecular Imaging"},{"key":"e_1_2_13_45_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0047-6374(00)00130-5"},{"key":"e_1_2_13_46_1","doi-asserted-by":"publisher","DOI":"10.3891\/acta.chem.scand.01-0944"},{"key":"e_1_2_13_47_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20112285"},{"key":"e_1_2_13_48_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nucmedbio.2011.02.016"},{"key":"e_1_2_13_49_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000001588"},{"key":"e_1_2_13_50_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.freeradbiomed.2011.10.446"},{"key":"e_1_2_13_51_1","doi-asserted-by":"publisher","DOI":"10.1039\/D0CP05744D"},{"key":"e_1_2_13_52_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M603337200"},{"key":"e_1_2_13_53_1","doi-asserted-by":"publisher","DOI":"10.1016\/B978-0-444-59565-2.00045-9"},{"key":"e_1_2_13_54_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa070613"},{"key":"e_1_2_13_55_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-007-0308-4"},{"key":"e_1_2_13_56_1","doi-asserted-by":"publisher","DOI":"10.1007\/s004010000355"},{"key":"e_1_2_13_57_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00687373"},{"key":"e_1_2_13_58_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1750-3639.1999.tb00549.x"},{"key":"e_1_2_13_59_1","doi-asserted-by":"publisher","DOI":"10.1021\/ic980730w"},{"key":"e_1_2_13_60_1","first-page":"119","article-title":"Progressive supranuclear palsy\u2014parkinsonian disorder with tau pathology","volume":"42","author":"Kowalska A.","year":"2004","journal-title":"Folia Neuropathologica"},{"key":"e_1_2_13_61_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtemb.2013.09.006"},{"key":"e_1_2_13_62_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.26512"},{"key":"e_1_2_13_63_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0006-8993(96)00782-2"},{"key":"e_1_2_13_64_1","doi-asserted-by":"publisher","DOI":"10.1080\/10715760100300651"},{"key":"e_1_2_13_65_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1749-6632.2009.05109.x"},{"key":"e_1_2_13_66_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-510X(98)00092-6"},{"key":"e_1_2_13_67_1","doi-asserted-by":"publisher","DOI":"10.1002\/cplu.201900504"},{"key":"e_1_2_13_68_1","doi-asserted-by":"publisher","DOI":"10.1002\/bip.20335"},{"key":"e_1_2_13_69_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.21170"},{"key":"e_1_2_13_70_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ab.2013.07.015"},{"key":"e_1_2_13_71_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(18)63504-5"},{"key":"e_1_2_13_72_1","doi-asserted-by":"publisher","DOI":"10.1021\/acschemneuro.8b00161"},{"key":"e_1_2_13_73_1","doi-asserted-by":"publisher","DOI":"10.1002\/ncp.10582"},{"key":"e_1_2_13_74_1","unstructured":"NCT00471211Study Evaluating the Safety Tolerability and Efficacy of PBT2 in Patients With Early Alzheimer's Disease."},{"key":"e_1_2_13_75_1","unstructured":"NCT02655315Conservative Iron Chelation as a Disease\u2010modifying Strategy in Parkinson's Disease (FAIRPARKII)."},{"key":"e_1_2_13_76_1","unstructured":"NCT03204929A Phase 1 Dose Escalation Study of Cu(II)ATSM Administered Orally to Patients With Early Idiopathic Parkinson's Disease."},{"key":"e_1_2_13_77_1","unstructured":"NCT03293069Conservative Iron Chelation as a Disease\u2010modifying Strategy in Amyotrophic Lateral Sclerosis (FAIR\u2010ALS II)."},{"key":"e_1_2_13_78_1","unstructured":"NCT04082832A Multicenter Randomized Double\u2010Blind Placebo\u2010Controlled Phase 2 Study of Cu(II)ATSM in Patients With Amyotrophic Lateral Sclerosis\/Motor Neuron Disease."},{"key":"e_1_2_13_79_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00571500"},{"key":"e_1_2_13_80_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-016-8169-1"},{"key":"e_1_2_13_81_1","doi-asserted-by":"publisher","DOI":"10.3390\/antiox11051022"},{"key":"e_1_2_13_82_1","doi-asserted-by":"publisher","DOI":"10.1093\/jnen\/60.4.393"},{"key":"e_1_2_13_83_1","doi-asserted-by":"publisher","DOI":"10.1258\/002367790780866128"},{"key":"e_1_2_13_84_1","doi-asserted-by":"publisher","DOI":"10.1093\/jnen\/nlx110"},{"key":"e_1_2_13_85_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12311-008-0091-3"},{"key":"e_1_2_13_86_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12011-014-0093-6"},{"key":"e_1_2_13_87_1","first-page":"25","article-title":"Supranuclear ophthalmoplegia, pseudobulbar palsy, nuchal dystonia and dementia. A clinical report on eight cases of \"Heterogenous system degeneration\"","volume":"88","author":"Richardson J. C.","year":"1963","journal-title":"Transactions of the American Neurological Association"},{"key":"e_1_2_13_88_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.4196-13.2014"},{"key":"e_1_2_13_89_1","doi-asserted-by":"publisher","DOI":"10.1021\/bi00403a017"},{"key":"e_1_2_13_90_1","doi-asserted-by":"publisher","DOI":"10.1038\/362059a0"},{"key":"e_1_2_13_91_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1471-4159.1999.0720422.x"},{"key":"e_1_2_13_92_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410180503"},{"key":"e_1_2_13_93_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neurobiolaging.2021.08.011"},{"key":"e_1_2_13_94_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1471-4159.2000.0740270.x"},{"key":"e_1_2_13_95_1","doi-asserted-by":"publisher","DOI":"10.1039\/C8MT00153G"},{"key":"e_1_2_13_96_1","doi-asserted-by":"publisher","DOI":"10.1159\/000111445"},{"key":"e_1_2_13_97_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M111.274407"},{"key":"e_1_2_13_98_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40263-016-0347-2"},{"key":"e_1_2_13_99_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1964.00460160003001"},{"key":"e_1_2_13_100_1","doi-asserted-by":"publisher","DOI":"10.1016\/0167-4838(89)90134-9"},{"key":"e_1_2_13_101_1","doi-asserted-by":"publisher","DOI":"10.1002\/mdc3.13489"},{"key":"e_1_2_13_102_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00216-004-2697-8"},{"key":"e_1_2_13_103_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0161-813X(01)00050-X"},{"key":"e_1_2_13_104_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13024-019-0341-5"},{"key":"e_1_2_13_105_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-017-1726-6"},{"key":"e_1_2_13_106_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-017-1779-6"},{"key":"e_1_2_13_107_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awac165"},{"key":"e_1_2_13_108_1","doi-asserted-by":"publisher","DOI":"10.1002\/anie.202000451"},{"key":"e_1_2_13_109_1","doi-asserted-by":"publisher","DOI":"10.1017\/S0317167100029140"},{"key":"e_1_2_13_110_1","doi-asserted-by":"publisher","DOI":"10.1186\/2047-9158-3-24"},{"key":"e_1_2_13_111_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddg312"},{"key":"e_1_2_13_112_1","doi-asserted-by":"publisher","DOI":"10.1039\/c003875j"},{"key":"e_1_2_13_113_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2016.01.020"},{"key":"e_1_2_13_114_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.2008.05321.x"},{"key":"e_1_2_13_115_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nucmedbio.2011.08.008"},{"key":"e_1_2_13_116_1","doi-asserted-by":"publisher","DOI":"10.1111\/neup.12707"},{"key":"e_1_2_13_117_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.arr.2023.101931"},{"key":"e_1_2_13_118_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.peptides.2007.08.022"},{"key":"e_1_2_13_119_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1976.00500020039007"}],"container-title":["Journal of Neurochemistry"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/jnc.15978","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,21]],"date-time":"2023-10-21T06:09:24Z","timestamp":1697868564000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/jnc.15978"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,10,6]]},"references-count":118,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2023,11]]}},"alternative-id":["10.1111\/jnc.15978"],"URL":"https:\/\/doi.org\/10.1111\/jnc.15978","archive":["Portico"],"relation":{"has-review":[{"id-type":"doi","id":"10.1111\/JNC.15978\/v1\/review2","asserted-by":"object"},{"id-type":"doi","id":"10.1111\/JNC.15978\/v1\/review1","asserted-by":"object"},{"id-type":"doi","id":"10.1111\/JNC.15978\/v2\/review1","asserted-by":"object"},{"id-type":"doi","id":"10.1111\/JNC.15978\/v2\/decision1","asserted-by":"object"},{"id-type":"doi","id":"10.1111\/JNC.15978\/v2\/review2","asserted-by":"object"},{"id-type":"doi","id":"10.1111\/JNC.15978\/v1\/decision1","asserted-by":"object"},{"id-type":"doi","id":"10.1111\/JNC.15978\/v2\/response1","asserted-by":"object"}]},"ISSN":["0022-3042","1471-4159"],"issn-type":[{"value":"0022-3042","type":"print"},{"value":"1471-4159","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,10,6]]},"assertion":[{"value":"2023-05-16","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-09-08","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-10-06","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}