{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T15:32:48Z","timestamp":1786980768639,"version":"build-2736575974"},"reference-count":64,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,10,4]],"date-time":"2024-10-04T00:00:00Z","timestamp":1728000000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,10,4]],"date-time":"2024-10-04T00:00:00Z","timestamp":1728000000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Biometals"],"published-print":{"date-parts":[[2025,2]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Toxic milk (txJ) is an autosomal recessive mutation in the\n                    <jats:italic>Atp7b<\/jats:italic>\n                    gene in the C3H\/HeJ strain, observed at The Jackson Laboratory in Maine, USA. TxJ mice exhibit symptoms similar to those of human Wilson\u2019s disease (WD). The study aimed to verify organ involvement in a mouse model of WD. TxJ mice and control animals were sacrificed at 2, 4, 8, and 14\u00a0months of age. Total X-ray Fluorescence Spectroscopy (TXRF) was used to determine the elemental concentration in organs. Tissue chemical composition was measured by Fourier Transform Infrared Spectroscopy (FTIR). Additionally, hybrid mapping of FTIR and microXRF was performed. Elevated concentrations of Cu were observed in the liver, striatum, eye, heart, and duodenum of txJ mice across age groups. In the striatum of the oldest txJ mice, there was lower lipid content and a higher fraction of saturated fats. The secondary structure of striatum proteins was disturbed in txJ mice. In the livers of txJ mice, higher concentrations of saturated fats and disturbances in the secondary structure of proteins were observed. The concentration of neurofilaments was significantly higher in txJ serum. The distribution of Cu deposits in brains was uniform with no prevalence in any anatomic structure in either group, but significant protein structure changes were observed exclusively in the striatum of txJ. In this txJ animal model of WD, pathologic copper accumulation occurs in the duodenum, heart, and eye tissues. Increased copper concentration in the liver and brain results in increased saturated fat content and disturbances in secondary protein structure, leading to hepatic injury and neurodegeneration.\n                  <\/jats:p>","DOI":"10.1007\/s10534-024-00640-y","type":"journal-article","created":{"date-parts":[[2024,10,4]],"date-time":"2024-10-04T11:02:01Z","timestamp":1728039721000},"page":"103-121","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Effect of primary copper metabolism disturbance on elemental, protein, and lipid composition of the organs in Jackson toxic milk mouse"],"prefix":"10.1007","volume":"38","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0471-3350","authenticated-orcid":false,"given":"Krzysztof","family":"Hadrian","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8331-1917","authenticated-orcid":false,"given":"Magdalena","family":"Szczerbowska-Boruchowska","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Artur","family":"Sur\u00f3wka","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3694-4076","authenticated-orcid":false,"given":"Olga","family":"Ciepiela","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2670-9651","authenticated-orcid":false,"given":"Tomasz","family":"Litwin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5851-2856","authenticated-orcid":false,"given":"Adam","family":"Przyby\u0142kowski","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,10,4]]},"reference":[{"issue":"5","key":"640_CR1","doi-asserted-by":"publisher","first-page":"555","DOI":"10.1007\/s10534-005-5918-5","volume":"19","author":"KJ Allen","year":"2006","unstructured":"Allen KJ, Buck NE, Cheah DM et al (2006) Chronological changes in tissue copper, zinc and iron in the toxic milk mouse and effects of copper loading. Biometals 19(5):555\u2013564. https:\/\/doi.org\/10.1007\/s10534-005-5918-5","journal-title":"Biometals"},{"key":"640_CR2","doi-asserted-by":"publisher","DOI":"10.3390\/diagnostics13091554","author":"A Antos","year":"2023","unstructured":"Antos A, Cz\u0142onkowska A, Bembenek J et al (2023) Blood based biomarkers of central nervous system involvement in Wilson\u2019s disease. Diagnostics (Basel). https:\/\/doi.org\/10.3390\/diagnostics13091554","journal-title":"Diagnostics (Basel)"},{"key":"640_CR3","doi-asserted-by":"publisher","first-page":"101034","DOI":"10.1016\/j.ymgmr.2023.101034","volume":"38","author":"Z Beyzaei","year":"2024","unstructured":"Beyzaei Z, Mehrzadeh A, Hashemi N et al (2024) The mutation spectrum and ethnic distribution of Wilson disease, a review. Mol Genet Metab Rep 38:101034. https:\/\/doi.org\/10.1016\/j.ymgmr.2023.101034","journal-title":"Mol Genet Metab Rep"},{"issue":"1","key":"640_CR4","first-page":"152","volume":"93","author":"RTSH Bronson","year":"1995","unstructured":"Bronson RTSH, Davisson MT (1995) Acute cerebral neuronal necrosis in copper deficient offspring of female mice with the toxic milk mutation. Mouse Genome 93(1):152\u2013154","journal-title":"Mouse Genome"},{"issue":"9","key":"640_CR5","doi-asserted-by":"publisher","first-page":"1665","DOI":"10.1093\/hmg\/8.9.1665","volume":"8","author":"OI Buiakova","year":"1999","unstructured":"Buiakova OI, Xu J, Lutsenko S et al (1999) Null mutation of the murine ATP7B (Wilson disease) gene results in intracellular copper accumulation and late-onset hepatic nodular transformation. Hum Mol Genet 8(9):1665\u20131671. https:\/\/doi.org\/10.1093\/hmg\/8.9.1665","journal-title":"Hum Mol Genet"},{"key":"640_CR6","doi-asserted-by":"publisher","first-page":"19","DOI":"10.1016\/b978-0-444-63625-6.00003-3","volume":"142","author":"IJ Chang","year":"2017","unstructured":"Chang IJ, Hahn SH (2017) The genetics of Wilson disease. Handb Clin Neurol 142:19\u201334. https:\/\/doi.org\/10.1016\/b978-0-444-63625-6.00003-3","journal-title":"Handb Clin Neurol"},{"issue":"5","key":"640_CR7","doi-asserted-by":"publisher","first-page":"1099","DOI":"10.1002\/jimd.12418","volume":"44","author":"K Chevalier","year":"2021","unstructured":"Chevalier K, Benyounes N, Obadia MA et al (2021) Cardiac involvement in Wilson disease: review of the literature and description of three cases of sudden death. J Inherit Metab Dis 44(5):1099\u20131112. https:\/\/doi.org\/10.1002\/jimd.12418","journal-title":"J Inherit Metab Dis"},{"issue":"10","key":"640_CR8","doi-asserted-by":"publisher","first-page":"793","DOI":"10.1007\/s00335-001-3021-y","volume":"12","author":"V Coronado","year":"2001","unstructured":"Coronado V, Nanji M, Cox DW (2001) The Jackson toxic milk mouse as a model for copper loading. Mamm Genome 12(10):793\u2013795. https:\/\/doi.org\/10.1007\/s00335-001-3021-y","journal-title":"Mamm Genome"},{"issue":"1","key":"640_CR9","doi-asserted-by":"publisher","first-page":"281","DOI":"10.3233\/jad-181008","volume":"68","author":"J Depciuch","year":"2019","unstructured":"Depciuch J, Zawlik I, Skrzypa M et al (2019) FTIR spectroscopy of cerebrospinal fluid reveals variations in the lipid: protein ratio at different stages of Alzheimer\u2019s disease. J Alzheimers Dis 68(1):281\u2013293. https:\/\/doi.org\/10.3233\/jad-181008","journal-title":"J Alzheimers Dis"},{"issue":"4","key":"640_CR10","doi-asserted-by":"publisher","first-page":"1433","DOI":"10.1002\/cphy.c170045","volume":"8","author":"C Doguer","year":"2018","unstructured":"Doguer C, Ha JH, Collins JF (2018) Intersection of iron and copper metabolism in the mammalian intestine and liver. Compr Physiol 8(4):1433\u20131461. https:\/\/doi.org\/10.1002\/cphy.c170045","journal-title":"Compr Physiol"},{"issue":"5","key":"640_CR11","doi-asserted-by":"publisher","first-page":"638","DOI":"10.7150\/thno.14596","volume":"6","author":"Y Dong","year":"2016","unstructured":"Dong Y, Ni W, Chen WJ et al (2016) Spectrum and classification of ATP7B variants in a large cohort of Chinese patients with Wilson\u2019s disease guides genetic diagnosis. Theranostics 6(5):638\u2013649. https:\/\/doi.org\/10.7150\/thno.14596","journal-title":"Theranostics"},{"key":"640_CR12","doi-asserted-by":"publisher","first-page":"193","DOI":"10.1016\/j.jtemb.2014.05.007","volume":"31","author":"P Dusek","year":"2015","unstructured":"Dusek P, Roos PM, Litwin T et al (2015) The neurotoxicity of iron, copper and manganese in Parkinson\u2019s and Wilson\u2019s diseases. J Trace Elem Med Biol 31:193\u2013203. https:\/\/doi.org\/10.1016\/j.jtemb.2014.05.007","journal-title":"J Trace Elem Med Biol"},{"key":"640_CR13","doi-asserted-by":"publisher","DOI":"10.21037\/atm.2019.02.43","author":"P Dusek","year":"2019","unstructured":"Dusek P, Litwin T, Cz\u0142onkowska A (2019a) Neurologic impairment in Wilson disease. Ann Transl Med. https:\/\/doi.org\/10.21037\/atm.2019.02.43","journal-title":"Ann Transl Med"},{"issue":"Suppl 2","key":"640_CR14","doi-asserted-by":"publisher","first-page":"S64","DOI":"10.21037\/atm.2019.02.43","volume":"7","author":"P Dusek","year":"2019","unstructured":"Dusek P, Litwin T, Cz\u0142onkowska A (2019b) Neurologic impairment in Wilson disease. Ann Transl Med 7(Suppl 2):S64. https:\/\/doi.org\/10.21037\/atm.2019.02.43","journal-title":"Ann Transl Med"},{"issue":"Suppl 2","key":"640_CR15","doi-asserted-by":"publisher","first-page":"S62","DOI":"10.21037\/atm.2019.03.30","volume":"7","author":"K Dzie\u017cyc-Jaworska","year":"2019","unstructured":"Dzie\u017cyc-Jaworska K, Litwin T, Cz\u0142onkowska A (2019) Clinical manifestations of Wilson disease in organs other than the liver and brain. Ann Transl Med 7(Suppl 2):S62. https:\/\/doi.org\/10.21037\/atm.2019.03.30","journal-title":"Ann Transl Med"},{"key":"640_CR16","doi-asserted-by":"publisher","DOI":"10.3390\/ijms21239259","author":"G Gromadzka","year":"2020","unstructured":"Gromadzka G, Tarnacka B, Flaga A et al (2020) Copper dyshomeostasis in neurodegenerative diseases-therapeutic implications. Int J Mol Sci. https:\/\/doi.org\/10.3390\/ijms21239259","journal-title":"Int J Mol Sci"},{"issue":"4","key":"640_CR17","doi-asserted-by":"publisher","first-page":"269","DOI":"10.1023\/a:1016546223327","volume":"25","author":"Z Hlubock\u00e1","year":"2002","unstructured":"Hlubock\u00e1 Z, Marecek Z, Linhart A et al (2002) Cardiac involvement in Wilson disease. J Inherit Metab Dis 25(4):269\u2013277. https:\/\/doi.org\/10.1023\/a:1016546223327","journal-title":"J Inherit Metab Dis"},{"issue":"6","key":"640_CR18","doi-asserted-by":"publisher","first-page":"309","DOI":"10.1016\/j.trsl.2006.06.005","volume":"148","author":"G Hou","year":"2006","unstructured":"Hou G, Dick R, Zeng C et al (2006) Comparison of lowering copper levels with tetrathiomolybdate and zinc on mouse tumor and doxorubicin models. Transl Res 148(6):309\u2013314. https:\/\/doi.org\/10.1016\/j.trsl.2006.06.005","journal-title":"Transl Res"},{"issue":"2","key":"640_CR19","doi-asserted-by":"publisher","first-page":"423","DOI":"10.2353\/ajpath.2006.050312","volume":"168","author":"D Huster","year":"2006","unstructured":"Huster D, Finegold MJ, Morgan CT et al (2006) Consequences of copper accumulation in the livers of the Atp7b-\/- (Wilson disease gene) knockout mice. Am J Pathol 168(2):423\u2013434. https:\/\/doi.org\/10.2353\/ajpath.2006.050312","journal-title":"Am J Pathol"},{"key":"640_CR20","doi-asserted-by":"publisher","first-page":"5828056","DOI":"10.1155\/2017\/5828056","volume":"2017","author":"D Ju\u00e1rez-Rebollar","year":"2017","unstructured":"Ju\u00e1rez-Rebollar D, Rios C, Nava-Ru\u00edz C et al (2017) Metallothionein in brain disorders. Oxid Med Cell Longev 2017:5828056. https:\/\/doi.org\/10.1155\/2017\/5828056","journal-title":"Oxid Med Cell Longev"},{"issue":"2","key":"640_CR21","doi-asserted-by":"publisher","first-page":"726","DOI":"10.1111\/ene.14573","volume":"28","author":"SFA J\u00fanior","year":"2021","unstructured":"J\u00fanior SFA, Ventura N, Marchiori E (2021) Osmotic demyelination syndrome diagnosed radiologically during Wilson\u2019s disease investigation. Eur J Neurol 28(2):726\u2013728. https:\/\/doi.org\/10.1111\/ene.14573","journal-title":"Eur J Neurol"},{"issue":"2","key":"640_CR22","doi-asserted-by":"publisher","first-page":"219","DOI":"10.1038\/ng0696-219","volume":"13","author":"EJ Kelly","year":"1996","unstructured":"Kelly EJ, Palmiter RD (1996) A murine model of Menkes disease reveals a physiological function of metallothionein. Nat Genet 13(2):219\u2013222. https:\/\/doi.org\/10.1038\/ng0696-219","journal-title":"Nat Genet"},{"issue":"5","key":"640_CR23","doi-asserted-by":"publisher","first-page":"8004","DOI":"10.3390\/ijms15058004","volume":"15","author":"A Le","year":"2014","unstructured":"Le A, Shibata NM, French SW et al (2014) Characterization of timed changes in hepatic copper concentrations, methionine metabolism, gene expression, and global DNA methylation in the Jackson toxic milk mouse model of Wilson disease. Int J Mol Sci 15(5):8004\u20138023. https:\/\/doi.org\/10.3390\/ijms15058004","journal-title":"Int J Mol Sci"},{"issue":"5","key":"640_CR24","doi-asserted-by":"publisher","first-page":"385","DOI":"10.1136\/practneurol-2014-001056","volume":"15","author":"T Litwin","year":"2015","unstructured":"Litwin T, Langwi\u0144ska-Wo\u015bko E, Dzie\u017cyc K et al (2015) Sunflower cataract: do not forget Wilson\u2019s disease. Pract Neurol 15(5):385\u2013386. https:\/\/doi.org\/10.1136\/practneurol-2014-001056","journal-title":"Pract Neurol"},{"issue":"14","key":"640_CR25","doi-asserted-by":"publisher","first-page":"1141","DOI":"10.1177\/1535370218813988","volume":"243","author":"J Liu","year":"2018","unstructured":"Liu J, Chen C, Liu Y et al (2018) Trientine selectively delivers copper to the heart and suppresses pressure overload-induced cardiac hypertrophy in rats. Exp Biol Med 243(14):1141\u20131152. https:\/\/doi.org\/10.1177\/1535370218813988","journal-title":"Exp Biol Med"},{"issue":"6","key":"640_CR26","doi-asserted-by":"publisher","first-page":"634","DOI":"10.4254\/wjh.v13.i6.634","volume":"13","author":"A Lucena-Valera","year":"2021","unstructured":"Lucena-Valera A, Perez-Palacios D, Mu\u00f1oz-Hernandez R et al (2021) Wilson\u2019s disease: revisiting an old friend. World J Hepatol 13(6):634\u2013649. https:\/\/doi.org\/10.4254\/wjh.v13.i6.634","journal-title":"World J Hepatol"},{"issue":"6","key":"640_CR27","doi-asserted-by":"publisher","first-page":"261","DOI":"10.1016\/j.medcli.2022.12.016","volume":"160","author":"A Lucena-Valera","year":"2023","unstructured":"Lucena-Valera A, Ruz-Zafra P, Ampuero J (2023) Wilson\u2019s disease: overview. Med Clin (Barc) 160(6):261\u2013267. https:\/\/doi.org\/10.1016\/j.medcli.2022.12.016","journal-title":"Med Clin (Barc)"},{"key":"640_CR28","doi-asserted-by":"publisher","DOI":"10.1242\/jcs.240523","author":"S Lutsenko","year":"2021","unstructured":"Lutsenko S (2021) Dynamic and cell-specific transport networks for intracellular copper ions. J Cell Sci. https:\/\/doi.org\/10.1242\/jcs.240523","journal-title":"J Cell Sci"},{"issue":"8","key":"640_CR29","doi-asserted-by":"publisher","first-page":"531","DOI":"10.1093\/qjmed\/hcx094","volume":"110","author":"H Matsuura","year":"2017","unstructured":"Matsuura H, Murata A, Shimazaki T et al (2017) Kayser-Fleischer ring: Wilson\u2019s disease. QJM 110(8):531. https:\/\/doi.org\/10.1093\/qjmed\/hcx094","journal-title":"QJM"},{"key":"640_CR30","doi-asserted-by":"publisher","DOI":"10.3390\/ijms20235937","author":"TA Mazi","year":"2019","unstructured":"Mazi TA, Sarode GV, Czlonkowska A et al (2019) Dysregulated choline, methionine, and aromatic amino acid metabolism in patients with Wilson disease: exploratory metabolomic profiling and implications for hepatic and neurologic phenotypes. Int J Mol Sci. https:\/\/doi.org\/10.3390\/ijms20235937","journal-title":"Int J Mol Sci"},{"key":"640_CR31","doi-asserted-by":"publisher","first-page":"57","DOI":"10.1016\/b978-0-444-63625-6.00006-9","volume":"142","author":"V Medici","year":"2017","unstructured":"Medici V, Huster D (2017) Animal models of Wilson disease. Handb Clin Neurol 142:57\u201370. https:\/\/doi.org\/10.1016\/b978-0-444-63625-6.00006-9","journal-title":"Handb Clin Neurol"},{"issue":"4","key":"640_CR32","doi-asserted-by":"publisher","first-page":"379","DOI":"10.5603\/PJNNS.a2023.0053","volume":"57","author":"M Misztal","year":"2023","unstructured":"Misztal M, Cz\u0142onkowska A, Cudna A et al (2023) Impact of treatment on blood-brain barrier impairment in Wilson\u2019s disease. Neurol Neurochir Pol 57(4):379\u2013386. https:\/\/doi.org\/10.5603\/PJNNS.a2023.0053","journal-title":"Neurol Neurochir Pol"},{"issue":"3","key":"640_CR33","doi-asserted-by":"publisher","first-page":"249","DOI":"10.1016\/j.taap.2011.02.003","volume":"256","author":"AD Monnot","year":"2011","unstructured":"Monnot AD, Behl M, Ho S et al (2011) Regulation of brain copper homeostasis by the brain barrier systems: effects of Fe-overload and Fe-deficiency. Toxicol Appl Pharmacol 256(3):249\u2013257. https:\/\/doi.org\/10.1016\/j.taap.2011.02.003","journal-title":"Toxicol Appl Pharmacol"},{"issue":"1","key":"640_CR34","doi-asserted-by":"publisher","first-page":"110","DOI":"10.4103\/0028-3886.25146","volume":"54","author":"SK Narayan","year":"2006","unstructured":"Narayan SK, Kaveer N (2006) CNS demyelination due to hypocupremia in Wilson\u2019s disease from overzealous treatment. Neurol India 54(1):110\u2013111. https:\/\/doi.org\/10.4103\/0028-3886.25146","journal-title":"Neurol India"},{"issue":"42","key":"640_CR35","doi-asserted-by":"publisher","first-page":"32385","DOI":"10.1074\/jbc.M110.143826","volume":"285","author":"Y Nose","year":"2010","unstructured":"Nose Y, Wood LK, Kim BE et al (2010) Ctr1 is an apical copper transporter in mammalian intestinal epithelial cells in vivo that is controlled at the level of protein stability. J Biol Chem 285(42):32385\u201332392. https:\/\/doi.org\/10.1074\/jbc.M110.143826","journal-title":"J Biol Chem"},{"issue":"4","key":"640_CR36","doi-asserted-by":"publisher","first-page":"G1181","DOI":"10.1152\/ajpgi.00472.2006","volume":"292","author":"L Nyasae","year":"2007","unstructured":"Nyasae L, Bustos R, Braiterman L et al (2007) Dynamics of endogenous ATP7A (Menkes protein) in intestinal epithelial cells: copper-dependent redistribution between two intracellular sites. Am J Physiol Gastrointest Liver Physiol 292(4):G1181\u2013G1194. https:\/\/doi.org\/10.1152\/ajpgi.00472.2006","journal-title":"Am J Physiol Gastrointest Liver Physiol"},{"key":"640_CR37","doi-asserted-by":"publisher","DOI":"10.3390\/ijms25042402","author":"EV Ovchinnikova","year":"2024","unstructured":"Ovchinnikova EV, Garbuz MM, Ovchinnikova AA et al (2024) Epidemiology of Wilson\u2019s disease and pathogenic variants of the ATP7B gene leading to diversified protein disfunctions. Int J Mol Sci. https:\/\/doi.org\/10.3390\/ijms25042402","journal-title":"Int J Mol Sci"},{"issue":"5","key":"640_CR38","doi-asserted-by":"publisher","first-page":"600","DOI":"10.1007\/s11307-011-0532-0","volume":"14","author":"F Peng","year":"2012","unstructured":"Peng F, Lutsenko S, Sun X et al (2012a) Imaging copper metabolism imbalance in Atp7b (-\/-) knockout mouse model of Wilson\u2019s disease with PET-CT and orally administered 64CuCl2. Mol Imaging Biol 14(5):600\u2013607. https:\/\/doi.org\/10.1007\/s11307-011-0532-0","journal-title":"Mol Imaging Biol"},{"issue":"1","key":"640_CR39","doi-asserted-by":"publisher","first-page":"70","DOI":"10.1007\/s11307-011-0476-4","volume":"14","author":"F Peng","year":"2012","unstructured":"Peng F, Lutsenko S, Sun X et al (2012b) Positron emission tomography of copper metabolism in the Atp7b-\/- knock-out mouse model of Wilson\u2019s disease. Mol Imaging Biol 14(1):70\u201378. https:\/\/doi.org\/10.1007\/s11307-011-0476-4","journal-title":"Mol Imaging Biol"},{"issue":"1","key":"640_CR40","doi-asserted-by":"publisher","first-page":"168","DOI":"10.1053\/j.gastro.2017.09.019","volume":"154","author":"H Pierson","year":"2018","unstructured":"Pierson H, Muchenditsi A, Kim BE et al (2018) The function of ATPase copper transporter ATP7B in intestine. Gastroenterology 154(1):168\u201380.e5. https:\/\/doi.org\/10.1053\/j.gastro.2017.09.019","journal-title":"Gastroenterology"},{"key":"640_CR41","doi-asserted-by":"publisher","first-page":"77","DOI":"10.1016\/b978-0-444-63625-6.00008-2","volume":"142","author":"A Poujois","year":"2017","unstructured":"Poujois A, Mikol J, Woimant F (2017) Wilson disease: brain pathology. Handb Clin Neurol 142:77\u201389. https:\/\/doi.org\/10.1016\/b978-0-444-63625-6.00008-2","journal-title":"Handb Clin Neurol"},{"issue":"10","key":"640_CR42","doi-asserted-by":"publisher","first-page":"2037","DOI":"10.1007\/s11064-013-1111-3","volume":"38","author":"A Przyby\u0142kowski","year":"2013","unstructured":"Przyby\u0142kowski A, Gromadzka G, Wawer A et al (2013) Neurochemical and behavioral characteristics of toxic milk mice: an animal model of Wilson\u2019s disease. Neurochem Res 38(10):2037\u20132045. https:\/\/doi.org\/10.1007\/s11064-013-1111-3","journal-title":"Neurochem Res"},{"issue":"3","key":"640_CR43","doi-asserted-by":"publisher","first-page":"141","DOI":"10.1093\/oxfordjournals.jhered.a109751","volume":"74","author":"H Rauch","year":"1983","unstructured":"Rauch H (1983) Toxic milk, a new mutation affecting cooper metabolism in the mouse. J Hered 74(3):141\u2013141","journal-title":"J Hered"},{"issue":"4","key":"640_CR44","doi-asserted-by":"publisher","first-page":"356","DOI":"10.1111\/jnc.14323","volume":"146","author":"E Reed","year":"2018","unstructured":"Reed E, Lutsenko S, Bandmann O (2018) Animal models of Wilson disease. J Neurochem 146(4):356\u2013373. https:\/\/doi.org\/10.1111\/jnc.14323","journal-title":"J Neurochem"},{"key":"640_CR45","doi-asserted-by":"publisher","first-page":"55","DOI":"10.3233\/BSI-200189","volume":"8","author":"A Rohman","year":"2019","unstructured":"Rohman A, Windarsih A, Lukitaningsih E et al (2019) The use of FTIR and Raman spectroscopy in combination with chemometrics for analysis of biomolecules in biomedical fluids: a review. Biomed Spectrosc Imaging 8:55\u201371. https:\/\/doi.org\/10.3233\/BSI-200189","journal-title":"Biomed Spectrosc Imaging"},{"issue":"11","key":"640_CR46","doi-asserted-by":"publisher","first-page":"2421","DOI":"10.4103\/1673-5374.393107","volume":"19","author":"S Roy","year":"2024","unstructured":"Roy S, Lutsenko S (2024) Mechanism of Cu entry into the brain: many unanswered questions. Neural Regen Res 19(11):2421\u20132429. https:\/\/doi.org\/10.4103\/1673-5374.393107","journal-title":"Neural Regen Res"},{"issue":"1","key":"640_CR47","doi-asserted-by":"publisher","first-page":"84","DOI":"10.1186\/s12968-021-00760-1","volume":"23","author":"J Salatzki","year":"2021","unstructured":"Salatzki J, Mohr I, Heins J et al (2021) The impact of Wilson disease on myocardial tissue and function: a cardiovascular magnetic resonance study. J Cardiovasc Magn Reson 23(1):84. https:\/\/doi.org\/10.1186\/s12968-021-00760-1","journal-title":"J Cardiovasc Magn Reson"},{"issue":"12","key":"640_CR48","doi-asserted-by":"publisher","first-page":"1556","DOI":"10.1093\/clinchem\/20.12.1556","volume":"20","author":"KH Schosinsky","year":"1974","unstructured":"Schosinsky KH, Lehmann HP, Beeler MF (1974) Measurement of ceruloplasmin from its oxidase activity in serum by use of o-dianisidine dihydrochloride. Clin Chem 20(12):1556\u20131563. https:\/\/doi.org\/10.1093\/clinchem\/20.12.1556","journal-title":"Clin Chem"},{"issue":"17","key":"640_CR49","doi-asserted-by":"publisher","first-page":"1394","DOI":"10.21037\/atm-21-2264","volume":"9","author":"SM Schroeder","year":"2021","unstructured":"Schroeder SM, Matsukuma KE, Medici V (2021) Wilson disease and the differential diagnosis of its hepatic manifestations: a narrative review of clinical, laboratory, and liver histological features. Ann Transl Med 9(17):1394. https:\/\/doi.org\/10.21037\/atm-21-2264","journal-title":"Ann Transl Med"},{"issue":"1","key":"640_CR50","doi-asserted-by":"publisher","first-page":"263","DOI":"10.1093\/brain\/awab274","volume":"145","author":"S Shribman","year":"2021","unstructured":"Shribman S, Bocchetta M, Sudre CH et al (2021a) Neuroimaging correlates of brain injury in Wilson\u2019s disease: a multimodal, whole-brain MRI study. Brain 145(1):263\u2013275. https:\/\/doi.org\/10.1093\/brain\/awab274","journal-title":"Brain"},{"issue":"2","key":"640_CR51","doi-asserted-by":"publisher","first-page":"503","DOI":"10.1002\/mds.28333","volume":"36","author":"S Shribman","year":"2021","unstructured":"Shribman S, Heller C, Burrows M et al (2021b) Plasma neurofilament light as a biomarker of neurological involvement in Wilson\u2019s disease. Mov Disord 36(2):503\u2013508. https:\/\/doi.org\/10.1002\/mds.28333","journal-title":"Mov Disord"},{"issue":"12","key":"640_CR52","doi-asserted-by":"publisher","first-page":"2446","DOI":"10.1002\/mds.29229","volume":"37","author":"L Smolinski","year":"2022","unstructured":"Smolinski L, Ziemssen T, Akgun K et al (2022) Brain atrophy is substantially accelerated in neurological Wilson\u2019s disease: a longitudinal study. Mov Disord 37(12):2446\u20132451. https:\/\/doi.org\/10.1002\/mds.29229","journal-title":"Mov Disord"},{"issue":"1","key":"640_CR53","doi-asserted-by":"publisher","first-page":"103","DOI":"10.1002\/alz.12179","volume":"17","author":"PJ Snyder","year":"2021","unstructured":"Snyder PJ, Alber J, Alt C et al (2021) Retinal imaging in Alzheimer\u2019s and neurodegenerative diseases. Alzheimers Dement 17(1):103\u2013111. https:\/\/doi.org\/10.1002\/alz.12179","journal-title":"Alzheimers Dement"},{"issue":"3","key":"640_CR54","doi-asserted-by":"publisher","first-page":"321","DOI":"10.1007\/s007020050026","volume":"107","author":"HJ Stuerenburg","year":"2000","unstructured":"Stuerenburg HJ (2000) CSF copper concentrations, blood-brain barrier function, and coeruloplasmin synthesis during the treatment of Wilson\u2019s disease. J Neural Transm (Vienna) 107(3):321\u2013329. https:\/\/doi.org\/10.1007\/s007020050026","journal-title":"J Neural Transm (Vienna)"},{"key":"640_CR55","doi-asserted-by":"publisher","first-page":"231","DOI":"10.1016\/j.redox.2016.12.007","volume":"11","author":"B Sullivan","year":"2017","unstructured":"Sullivan B, Robison G, Osborn J et al (2017) On the nature of the Cu-rich aggregates in brain astrocytes. Redox Biol 11:231\u2013239. https:\/\/doi.org\/10.1016\/j.redox.2016.12.007","journal-title":"Redox Biol"},{"issue":"1","key":"640_CR56","doi-asserted-by":"publisher","first-page":"105","DOI":"10.1111\/j.1471-4159.2011.07278.x","volume":"118","author":"D Terwel","year":"2011","unstructured":"Terwel D, L\u00f6schmann YN, Schmidt HH et al (2011) Neuroinflammatory and behavioural changes in the Atp7B mutant mouse model of Wilson\u2019s disease. J Neurochem 118(1):105\u2013112. https:\/\/doi.org\/10.1111\/j.1471-4159.2011.07278.x","journal-title":"J Neurochem"},{"issue":"10","key":"640_CR57","doi-asserted-by":"publisher","first-page":"1619","DOI":"10.1093\/hmg\/5.10.1619","volume":"5","author":"MB Theophilos","year":"1996","unstructured":"Theophilos MB, Cox DW, Mercer JF (1996) The toxic milk mouse is a murine model of Wilson disease. Hum Mol Genet 5(10):1619\u20131624. https:\/\/doi.org\/10.1093\/hmg\/5.10.1619","journal-title":"Hum Mol Genet"},{"issue":"40","key":"640_CR58","doi-asserted-by":"publisher","first-page":"e21871","DOI":"10.1097\/md.0000000000021871","volume":"99","author":"H Wang","year":"2020","unstructured":"Wang H, Wang W, Shi H et al (2020a) Blood neurofilament light chain in Parkinson disease and atypical parkinsonisms: a protocol for systematic review and meta-analysis. Medicine 99(40):e21871. https:\/\/doi.org\/10.1097\/md.0000000000021871","journal-title":"Medicine"},{"key":"640_CR59","doi-asserted-by":"publisher","first-page":"103279","DOI":"10.1016\/j.infrared.2020.103279","volume":"106","author":"X Wang","year":"2020","unstructured":"Wang X, Wu Q, Li C et al (2020b) A study of Parkinson\u2019s disease patients\u2019 serum using FTIR spectroscopy. Infrared Phys Technol 106:103279. https:\/\/doi.org\/10.1016\/j.infrared.2020.103279","journal-title":"Infrared Phys Technol"},{"issue":"1","key":"640_CR60","doi-asserted-by":"publisher","first-page":"e1010558","DOI":"10.1371\/journal.pgen.1010558","volume":"19","author":"CL Washington-Hughes","year":"2023","unstructured":"Washington-Hughes CL, Roy S, Seneviratne HK et al (2023) Atp7b-dependent choroid plexus dysfunction causes transient copper deficit and metabolic changes in the developing mouse brain. PLoS Genet 19(1):e1010558. https:\/\/doi.org\/10.1371\/journal.pgen.1010558","journal-title":"PLoS Genet"},{"issue":"2","key":"640_CR61","doi-asserted-by":"publisher","first-page":"322","DOI":"10.1515\/cclm-2023-0518","volume":"62","author":"D Wilson","year":"2024","unstructured":"Wilson D, Chan D, Chang L et al (2024) Development and multi-center validation of a fully automated digital immunoassay for neurofilament light chain: toward a clinical blood test for neuronal injury. Clin Chem Lab Med 62(2):322\u2013331. https:\/\/doi.org\/10.1515\/cclm-2023-0518","journal-title":"Clin Chem Lab Med"},{"issue":"3","key":"640_CR62","doi-asserted-by":"publisher","first-page":"6419","DOI":"10.3390\/ijms16036419","volume":"16","author":"F Wu","year":"2015","unstructured":"Wu F, Wang J, Pu C et al (2015) Wilson\u2019s disease: a comprehensive review of the molecular mechanisms. Int J Mol Sci 16(3):6419\u20136431. https:\/\/doi.org\/10.3390\/ijms16036419","journal-title":"Int J Mol Sci"},{"key":"640_CR63","doi-asserted-by":"publisher","first-page":"911882","DOI":"10.3389\/fneur.2022.911882","volume":"13","author":"ZW Zheng","year":"2022","unstructured":"Zheng ZW, Xu MH, Sun CB et al (2022) Acute-onset visual impairment in Wilson\u2019s disease: a case report and literature review. Front Neurol 13:911882. https:\/\/doi.org\/10.3389\/fneur.2022.911882","journal-title":"Front Neurol"},{"issue":"12","key":"640_CR64","doi-asserted-by":"publisher","first-page":"e01459","DOI":"10.1002\/brb3.1459","volume":"9","author":"X-x Zhou","year":"2019","unstructured":"Zhou X-x, Li X-h, Chen D-b et al (2019) Injury factors and pathological features of toxic milk mice during different disease stages. Brain Behav 9(12):e01459. https:\/\/doi.org\/10.1002\/brb3.1459","journal-title":"Brain Behav"}],"container-title":["BioMetals"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10534-024-00640-y.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s10534-024-00640-y\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10534-024-00640-y.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,1,22]],"date-time":"2025-01-22T05:35:29Z","timestamp":1737524129000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s10534-024-00640-y"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,10,4]]},"references-count":64,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2025,2]]}},"alternative-id":["640"],"URL":"https:\/\/doi.org\/10.1007\/s10534-024-00640-y","relation":{},"ISSN":["0966-0844","1572-8773"],"issn-type":[{"value":"0966-0844","type":"print"},{"value":"1572-8773","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,10,4]]},"assertion":[{"value":"17 July 2024","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"19 September 2024","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"4 October 2024","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The authors have no relevant financial or nonfinancial interests to disclose.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"This is an observational study. The first Local Bioethics Committee for Animal Research in Warsaw, Poland has confirmed that no ethical approval is required.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}}]}}