{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,4]],"date-time":"2026-07-04T10:20:04Z","timestamp":1783160404260,"version":"3.54.6"},"reference-count":92,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2026,6,24]],"date-time":"2026-06-24T00:00:00Z","timestamp":1782259200000},"content-version":"vor","delay-in-days":174,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Italian Ministry of Health Ricerca Corrente","award":["(PNRR)-MCNT1-2023-12376993"],"award-info":[{"award-number":["(PNRR)-MCNT1-2023-12376993"]}]},{"DOI":"10.13039\/501100003196","name":"Italian Ministry of Health","doi-asserted-by":"publisher","award":["PNRR-POC (Proof of Concept)-2023-12377653"],"award-info":[{"award-number":["PNRR-POC (Proof of Concept)-2023-12377653"]}],"id":[{"id":"10.13039\/501100003196","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003196","name":"Italian Ministry of Health","doi-asserted-by":"publisher","award":["PNRR-POC-2022-12375645"],"award-info":[{"award-number":["PNRR-POC-2022-12375645"]}],"id":[{"id":"10.13039\/501100003196","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Italian Ministry of Education and Research"},{"DOI":"10.13039\/100012352","name":"University of Milan","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100012352","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100015229","name":"PNC","doi-asserted-by":"publisher","award":["PNC-E3-2022-23683266\u2013 CUP: C43C22001630001"],"award-info":[{"award-number":["PNC-E3-2022-23683266\u2013 CUP: C43C22001630001"]}],"id":[{"id":"10.13039\/100015229","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,7,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Amyotrophic lateral sclerosis is a progressive neurodegenerative disease characterized by accumulation of the 43-kDa TAR DNA-binding protein (TDP-43). This neuropathological signature has been well documented within the CNS; however, recent findings indicate that the phosphorylated TDP-43 additionally deposits in peripheral tissues, including skeletal muscle and intramuscular nerves. These data warrant a change of view from a neurocentric perspective of amyotrophic lateral sclerosis pathogenesis towards a broader concept of TDP-43 proteinopathy extending both within and beyond the nervous system.<\/jats:p>\n                  <jats:p>In this review, we focus on current evidence supporting the presence of TDP-43 pathology in amyotrophic lateral sclerosis skeletal muscle, examining its topographic distribution, molecular characteristics and associations with intramuscular nerve bundles. We also discuss the susceptibility of intrinsic muscle cells, disrupted axonal transport and impairment in protein quality control. Phosphorylated TDP-43 pathology in muscle biopsies from amyotrophic lateral sclerosis patients has emerged as a promising tool in the early diagnosis of the disease. Moreover, we discuss the relevance of these findings to amyotrophic lateral sclerosis pathogenesis and potential therapeutic implications.<\/jats:p>","DOI":"10.1093\/braincomms\/fcag241","type":"journal-article","created":{"date-parts":[[2026,6,22]],"date-time":"2026-06-22T11:22:22Z","timestamp":1782127342000},"source":"Crossref","is-referenced-by-count":0,"title":["Beyond motor neurons: peripheral TDP-43 pathology in skeletal muscle and intramuscular nerves in amyotrophic lateral sclerosis"],"prefix":"10.1093","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5425-969X","authenticated-orcid":false,"given":"Stefania","family":"Corti","sequence":"first","affiliation":[{"name":"Dino Ferrari Center, Department of Pathophysiology and Transplantation (DEPT), University of Milan , Milan 20122 ,","place":["Italy"]},{"name":"Neuromuscular and Rare Diseases Unit, Department of Neurosciences, Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico , Milan 20122 ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudia","family":"Alberti","sequence":"additional","affiliation":[{"name":"Dino Ferrari Center, Department of Pathophysiology and Transplantation (DEPT), University of Milan , Milan 20122 ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Linda","family":"Ottoboni","sequence":"additional","affiliation":[{"name":"Dino Ferrari Center, Department of Pathophysiology and Transplantation (DEPT), University of Milan , Milan 20122 ,","place":["Italy"]},{"name":"Neurology Unit, Department of Neurosciences, Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico , Milan 20122 ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulia","family":"Magni","sequence":"additional","affiliation":[{"name":"Neurology Unit, Department of Neurosciences, Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico , Milan 20122 ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8460-938X","authenticated-orcid":false,"given":"Delia","family":"Gagliardi","sequence":"additional","affiliation":[{"name":"Neurology Unit, Department of Neurosciences, Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico , Milan 20122 ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Filippo","family":"Marcotti","sequence":"additional","affiliation":[{"name":"Neurology Unit, Department of Neurosciences, Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico , Milan 20122 ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simona","family":"Zanotti","sequence":"additional","affiliation":[{"name":"Neuromuscular and Rare Diseases Unit, Department of Neurosciences, Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico , Milan 20122 ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maurizio","family":"Moggio","sequence":"additional","affiliation":[{"name":"Neuromuscular and Rare Diseases Unit, Department of Neurosciences, Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico , Milan 20122 ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giacomo Pietro","family":"Comi","sequence":"additional","affiliation":[{"name":"Dino Ferrari Center, Department of Pathophysiology and Transplantation (DEPT), University of Milan , Milan 20122 ,","place":["Italy"]},{"name":"Neurology Unit, Department of Neurosciences, Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico , Milan 20122 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