{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,26]],"date-time":"2026-10-26T18:56:32Z","timestamp":1793040992868,"version":"4.5.0"},"reference-count":112,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,4]]},"abstract":"<jats:p>The onset of amyotrophic lateral sclerosis is usually characterized by focal death of both upper and\/or lower motor neurons occurring in the motor cortex, basal ganglia, brainstem, and spinal cord, and commonly involves the muscles of the upper and\/or lower extremities, and the muscles of the bulbar and\/or respiratory regions. However, as the disease progresses, it affects the adjacent body regions, leading to generalized muscle weakness, occasionally along with memory, cognitive, behavioral, and language impairments; respiratory dysfunction occurs at the final stage of the disease. The disease has a complicated pathophysiology and currently, only riluzole, edaravone, and phenylbutyrate\/taurursodiol are licensed to treat amyotrophic lateral sclerosis in many industrialized countries. The TAR DNA-binding protein 43 inclusions are observed in 97% of those diagnosed with amyotrophic lateral sclerosis. This review provides a preliminary overview of the potential effects of TAR DNA-binding protein 43 in the pathogenesis of amyotrophic lateral sclerosis, including the abnormalities in nucleoplasmic transport, RNA function, post-translational modification, liquid-liquid phase separation, stress granules, mitochondrial dysfunction, oxidative stress, axonal transport, protein quality control system, and non-cellular autonomous functions (e.g., glial cell functions and prion-like propagation).<\/jats:p>","DOI":"10.4103\/1673-5374.382233","type":"journal-article","created":{"date-parts":[[2023,9,9]],"date-time":"2023-09-09T11:02:28Z","timestamp":1694257348000},"page":"800-806","source":"Crossref","is-referenced-by-count":35,"title":["The pathogenic mechanism of TAR DNA-binding protein 43 (TDP-43) in amyotrophic lateral sclerosis"],"prefix":"10.4103","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1762-6689","authenticated-orcid":false,"given":"Xinxin","family":"Wang","sequence":"first","affiliation":[{"name":"Medical College of Nanchang University, Nanchang, Jiangxi Province, China"},{"name":"Department of Neurology, Jiangxi Provincial People\u2019s Hospital, The First Affiliated Hospital of Nanchang Medical College, The Clinical College of Nanchang Medical College, Nanchang, Jiangxi Province, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7315-5036","authenticated-orcid":false,"given":"Yushu","family":"Hu","sequence":"additional","affiliation":[{"name":"Medical College of Nanchang University, Nanchang, Jiangxi Province, China"},{"name":"Department of Neurology, Jiangxi Provincial People\u2019s Hospital, The First Affiliated Hospital of Nanchang Medical College, The Clinical College of Nanchang Medical College, Nanchang, Jiangxi Province, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0313-3434","authenticated-orcid":false,"given":"Renshi","family":"Xu","sequence":"additional","affiliation":[{"name":"Medical College of Nanchang University, Nanchang, Jiangxi Province, China"},{"name":"Department of Neurology, Jiangxi Provincial People\u2019s Hospital, The First Affiliated Hospital of Nanchang Medical College, The Clinical College of Nanchang Medical College, Nanchang, Jiangxi Province, China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2023,9,4]]},"reference":[{"key":"R1-20230909","doi-asserted-by":"crossref","first-page":"536","DOI":"10.1016\/j.neuron.2013.12.018","article-title":"Axonal transport of TDP-43 mRNA granules is impaired by ALS-causing 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