{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T07:12:50Z","timestamp":1791529970415,"version":"4.3.4"},"reference-count":10,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2025,6,9]],"date-time":"2025-06-09T00:00:00Z","timestamp":1749427200000},"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":["Muscle and Nerve"],"published-print":{"date-parts":[[2025,9]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Introduction\/Aims<\/jats:title><jats:p>Cardiac troponin T (cTnT) levels are elevated in the majority of persons with amyotrophic lateral sclerosis (ALS) and increase over time. Neurofilament light chain (NfL) is an established therapy response biomarker in ALS as superoxide dismutase1 (<jats:italic>SOD1<\/jats:italic>)\u2010ALS patients treated with the antisense oligonucleotide tofersen show a decrease in NfL. In this study, we assess cTnT levels in <jats:italic>SOD1<\/jats:italic>\u2010ALS at baseline and during tofersen treatment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>cTnT was analyzed at baseline and during tofersen treatment in 23 <jats:italic>SOD1<\/jats:italic>\u2010ALS patients at two specialized ALS centers in Germany and compared to a control cohort of 74 ALS patients without <jats:italic>SOD1<\/jats:italic> variants.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>cTnT levels increased in the control ALS cohort over time (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.0001) but not in the tofersen group (<jats:italic>p<\/jats:italic>\u2009=\u20090.36). Creatine kinase (CK) and CK\u2010MB levels did not show significant changes over time. The median monthly increase of cTnT was 0.045 points (IQR 0.02\u20130.08) in the control ALS cohort and 0.01 points (IQR \u22120.01\u20130.03) in the tofersen group (<jats:italic>p<\/jats:italic>\u2009=\u20090.0013). A significantly lower fold change in cTnT levels was observed in the tofersen\u2010treated cohort (median 1.2; IQR 0.77\u20131.59) relative to the control group (median 1.89; IQR 1.35\u20132.75) (<jats:italic>p<\/jats:italic>\u2009=\u20090.0003). Nine (39%) patients treated with tofersen experienced a reduction in cTnT levels.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>In this study, we describe a response signal of cTnT to tofersen treatment, which supports the value of cTnT as an independent biomarker in ALS. These results contribute to the notion that cTnT may provide additional value as a progression and treatment response biomarker in ALS complementary to NfL and warrant further investigation.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.28453","type":"journal-article","created":{"date-parts":[[2025,6,10]],"date-time":"2025-06-10T02:59:28Z","timestamp":1749524368000},"page":"509-514","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Serum Cardiac Troponin T Levels as a Therapy Response Marker in Tofersen\u2010Treated <scp>ALS<\/scp>"],"prefix":"10.1002","volume":"72","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7485-2626","authenticated-orcid":false,"given":"Sarah","family":"Bernsen","sequence":"first","affiliation":[{"name":"Department of Neuromuscular Diseases, Center for Neurology University Hospital Bonn  Bonn Germany"},{"name":"Deutsches Zentrum f\u00fcr Neurodegenerative Erkrankungen, Research Site Bonn  Bonn Germany"},{"name":"Department of Neurology, Center for ALS and Other Motor Neuron Disorders, Charit\u00e9\u2014Universit\u00e4tsmedizin Berlin, Corporate Member of Freie Universit\u00e4t Berlin Humboldt\u2010Universit\u00e4t Zu Berlin and Berlin Institute of Health  Berlin Germany"},{"name":"Department of Parkinson, Sleep and Movement Disorders, Center for Neurology University Hospital Bonn  Bonn Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rachel","family":"Fabian","sequence":"additional","affiliation":[{"name":"Department of Neuromuscular Diseases, Center for Neurology University Hospital Bonn  Bonn Germany"},{"name":"Department of Parkinson, Sleep and Movement Disorders, Center for Neurology University Hospital Bonn  Bonn Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasemin","family":"Koc","sequence":"additional","affiliation":[{"name":"Department of Neurology, Center for ALS and Other Motor Neuron Disorders, Charit\u00e9\u2014Universit\u00e4tsmedizin Berlin, Corporate Member of Freie Universit\u00e4t Berlin Humboldt\u2010Universit\u00e4t Zu Berlin and Berlin Institute of Health  Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peggy","family":"Schumann","sequence":"additional","affiliation":[{"name":"Department of Neurology, Center for ALS and Other Motor Neuron Disorders, Charit\u00e9\u2014Universit\u00e4tsmedizin Berlin, Corporate Member of Freie Universit\u00e4t Berlin Humboldt\u2010Universit\u00e4t Zu Berlin and Berlin Institute of Health  Berlin Germany"},{"name":"Ambulanzpartner Soziotechnologie APST GmbH  Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"K\u00f6rtv\u00e9lyessy","sequence":"additional","affiliation":[{"name":"Department of Neurology, Center for ALS and Other Motor Neuron Disorders, Charit\u00e9\u2014Universit\u00e4tsmedizin Berlin, Corporate Member of Freie Universit\u00e4t Berlin Humboldt\u2010Universit\u00e4t Zu Berlin and Berlin Institute of Health  Berlin Germany"},{"name":"Deutsches Zentrum f\u00fcr Neurodegenerative Erkrankungen, Research Site Magdeburg  Magdeburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1581-474X","authenticated-orcid":false,"given":"Sergio","family":"Castro\u2010Gomez","sequence":"additional","affiliation":[{"name":"Department of Parkinson, Sleep and Movement Disorders, Center for Neurology University Hospital Bonn  Bonn Germany"},{"name":"Institute of Physiology II, University Hospital Bonn  Bonn Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2736-7350","authenticated-orcid":false,"given":"Thomas","family":"Meyer","sequence":"additional","affiliation":[{"name":"Department of Neurology, Center for ALS and Other Motor Neuron Disorders, Charit\u00e9\u2014Universit\u00e4tsmedizin Berlin, Corporate Member of Freie Universit\u00e4t Berlin Humboldt\u2010Universit\u00e4t Zu Berlin and Berlin Institute of Health  Berlin Germany"},{"name":"Ambulanzpartner Soziotechnologie APST GmbH  Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick","family":"Weydt","sequence":"additional","affiliation":[{"name":"Department of Neuromuscular Diseases, Center for Neurology University Hospital Bonn  Bonn Germany"},{"name":"Deutsches Zentrum f\u00fcr Neurodegenerative Erkrankungen, Research Site Bonn  Bonn Germany"},{"name":"Department of Parkinson, Sleep and Movement Disorders, Center for Neurology University Hospital Bonn  Bonn Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,6,9]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa2204705"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.27818"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2014.03.027"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/acn3.52126"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1093\/braincomms\/fcab274"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1080\/21678421.2021.1939384"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.3109\/17482960903093710"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000194316.91908.8a"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijms241411682"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.28182"}],"container-title":["Muscle &amp; Nerve"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.28453","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,11]],"date-time":"2025-08-11T14:14:28Z","timestamp":1754921668000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/mus.28453"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,6,9]]},"references-count":10,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2025,9]]}},"alternative-id":["10.1002\/mus.28453"],"URL":"https:\/\/doi.org\/10.1002\/mus.28453","archive":["Portico"],"relation":{},"ISSN":["0148-639X","1097-4598"],"issn-type":[{"value":"0148-639X","type":"print"},{"value":"1097-4598","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,6,9]]},"assertion":[{"value":"2025-02-15","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-05-31","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-06-09","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}