{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,28]],"date-time":"2026-07-28T00:41:23Z","timestamp":1785199283812,"version":"3.55.0"},"reference-count":20,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2020,8,9]],"date-time":"2020-08-09T00:00:00Z","timestamp":1596931200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2020,12]]},"abstract":"<jats:p>We report for the first time the presence of phosphorylated transactivation response DNA\u2010binding protein of 43\u2009kDa (p\u2010TDP\u201043)\u2010immunoreactive cytoplasmic inclusions in Schwann cells in an autopsy case of sporadic amyotrophic lateral sclerosis (ALS). An 81\u2010year\u2010old woman with no family history of neuromuscular disorders noticed difficulty in handling chopsticks due to weakness of the hands. She then developed weakness of the lower and upper limbs and dyspnea. Neurological examination at the age of 83\u2009years revealed disorientation, severe weakness of the facial muscles, tongue, neck and extremities, and fasciculations in the thighs. She exhibited hyperactive jaw jerk and lower limb deep tendon reflexes and normal upper limb deep tendon reflexes, and left extensor plantar response was observed. The patient was diagnosed as having sporadic ALS. An autopsy performed at the age of 84\u2009years revealed widespread p\u2010TDP\u201043\u2010immunoreactive neuronal and glial cytoplasmic inclusions in the cerebrum, brain stem, and spinal cord, in addition to some Bunina bodies. Moreover, a small number of p\u2010TDP\u201043\u2010immunoreactive inclusions were found in the facial or accoustic nerve (indistinguishable), spinal cord anterior roots, cauda equina, and peripheral nerves in the dorsal root ganglia. Immunohistochemical staining for p\u2010TDP\u201043 revealed just a few p\u2010TDP\u201043\u2010immunoreactive inclusions surrounding axons in the cervical and lumbar anterior roots. Double immunofluorescence analysis revealed that these inclusions were co\u2010localized with S\u2010100 protein \u03b2, suggesting that these inclusions were localized in the cytoplasm of Schwann cells. The peripheral nervous system including Schwann cells may be involved in TDP\u201043 pathology in ALS.<\/jats:p>","DOI":"10.1111\/neup.12673","type":"journal-article","created":{"date-parts":[[2020,8,10]],"date-time":"2020-08-10T00:19:31Z","timestamp":1597018771000},"page":"606-610","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Accumulation of phosphorylated <scp>TDP<\/scp>\u201043 in the cytoplasm of Schwann cells in a case of sporadic amyotrophic lateral sclerosis"],"prefix":"10.1111","volume":"40","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4964-669X","authenticated-orcid":false,"given":"Keiko","family":"Nakamura\u2010Shindo","sequence":"first","affiliation":[{"name":"Department of Neurology and Neurobiology of Aging Kanazawa University Graduate School of Medical Sciences  Kanazawa Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8865-4097","authenticated-orcid":false,"given":"Kenji","family":"Sakai","sequence":"additional","affiliation":[{"name":"Department of Neurology and Neurobiology of Aging Kanazawa University Graduate School of Medical Sciences  Kanazawa Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ai","family":"Shimizu","sequence":"additional","affiliation":[{"name":"Department of Neurology and Neurobiology of Aging Kanazawa University Graduate School of Medical Sciences  Kanazawa Japan"},{"name":"Department of Neurology, Hokuriku Brain and Neuromuscular Disease Center National Hospital Organization Iou National Hospital  Kanazawa Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1948-5381","authenticated-orcid":false,"given":"Chiho","family":"Ishida","sequence":"additional","affiliation":[{"name":"Department of Neurology, Hokuriku Brain and Neuromuscular Disease Center National Hospital Organization Iou National Hospital  Kanazawa Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masahito","family":"Yamada","sequence":"additional","affiliation":[{"name":"Department of Neurology and Neurobiology of Aging Kanazawa University Graduate School of Medical Sciences  Kanazawa Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2020,8,9]]},"reference":[{"key":"e_1_2_7_2_1","first-page":"817","volume-title":"Greenfield's Neuropathology","author":"Ince PG","year":"2015"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1128\/jvi.69.6.3584-3596.1995"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1134108"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-008-0385-z"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.21425"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.jnen.0000174335.27708.0a"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbrc.2006.10.093"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-018-8897-5"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinph.2007.09.143"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.febslet.2008.07.027"},{"key":"e_1_2_7_12_1","unstructured":"TDP\u201043 Antibody.Proteintech\u00ae. 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