{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,7]],"date-time":"2026-08-07T14:36:52Z","timestamp":1786113412270,"version":"build-2736575974"},"reference-count":28,"publisher":"Oxford University Press (OUP)","issue":"8","license":[{"start":{"date-parts":[[2026,4,2]],"date-time":"2026-04-02T00:00:00Z","timestamp":1775088000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"JSPS KAKENHI","award":["23K06802"],"award-info":[{"award-number":["23K06802"]}]},{"name":"JSPS KAKENHI","award":["25K10784"],"award-info":[{"award-number":["25K10784"]}]},{"name":"JSPS KAKENHI","award":["24K10654"],"award-info":[{"award-number":["24K10654"]}]},{"name":"JSPS KAKENHI","award":["23K24209"],"award-info":[{"award-number":["23K24209"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,8,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Promyelocytic leukemia protein nuclear bodies (PML-NBs) and stress granules serve as deposition sites for stress-induced, aggregation-prone proteins. We previously reported that TAR DNA-binding protein 43 (TDP-43) colocalizes with stress granules during early aggregation in sporadic amyotrophic lateral sclerosis (ALS), and recent studies have noted PML-NB loss in familial ALS. To explore the role of PML-NBs in TDP-43 inclusion maturation, we analyzed spinal cord specimens from 12 patients with sporadic ALS and 5 controls using immunostaining for PML and TDP-43. PML-NB counts in anterior horn cells (AHCs) were significantly lower in patients with ALS than in controls (P\u202f&amp;lt;\u202f0.05), especially in AHCs with TDP-43 inclusions (P\u202f&amp;lt;\u202f0.01). Average numbers of PML-NB decreased progressively with inclusion type (3.1 in diffuse punctate cytoplasmic staining, 2.3 in round inclusions, and 0.8 in skein-like inclusions); all of these were significantly lower than those in inclusion-free AHCs (controls: 4.6; ALS: 5.5; P\u202f&amp;lt;\u202f0.01). AHCs in ALS without inclusions showed higher PML-NB counts than in controls (P\u202f&amp;lt;\u202f0.05), suggesting an early protective response. In contrast, reduced PML-NBs in mature inclusions may reflect diminished cellular defense. These findings implicate PML-NBs in the pathogenesis of sporadic ALS.<\/jats:p>","DOI":"10.1093\/jnen\/nlag029","type":"journal-article","created":{"date-parts":[[2026,3,18]],"date-time":"2026-03-18T12:54:42Z","timestamp":1773838482000},"page":"903-912","source":"Crossref","is-referenced-by-count":0,"title":["Relationship between promyelocytic leukemia protein nuclear bodies and TAR DNA-binding protein-43 aggregation in spinal anterior horn cells in sporadic amyotrophic lateral sclerosis"],"prefix":"10.1093","volume":"85","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1903-1766","authenticated-orcid":false,"given":"Fumiaki","family":"Mori","sequence":"first","affiliation":[{"name":"Department of Neuropathology, Biomedical Research Center, Hirosaki University Graduate School of Medicine , Hirosaki,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6072-3954","authenticated-orcid":false,"given":"Tomoya","family":"Kon","sequence":"additional","affiliation":[{"name":"Department of Neurology, Hirosaki University Graduate School of Medicine , Hirosaki,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Riho","family":"Itazawa","sequence":"additional","affiliation":[{"name":"Department of Neurology, Hirosaki University Graduate School of Medicine , Hirosaki,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ami","family":"Akatsu","sequence":"additional","affiliation":[{"name":"Department of Neuropathology, Biomedical Research Center, Hirosaki University Graduate School of Medicine , Hirosaki,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9375-9590","authenticated-orcid":false,"given":"Yasuo","family":"Miki","sequence":"additional","affiliation":[{"name":"Department of Neuropathology, Biomedical Research Center, Hirosaki University Graduate School of Medicine , Hirosaki,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akira","family":"Arai","sequence":"additional","affiliation":[{"name":"Department of Neurology, Aomori Prefectural Central Hospital , Aomori,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hidekachi","family":"Kurotaki","sequence":"additional","affiliation":[{"name":"Department of Pathology, Aomori Prefectural Central Hospital , Aomori,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masahiko","family":"Tomiyama","sequence":"additional","affiliation":[{"name":"Department of Neurology, Hirosaki University Graduate School of Medicine , Hirosaki,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Koichi","family":"Wakabayashi","sequence":"additional","affiliation":[{"name":"Department of Neuropathology, Biomedical Research Center, Hirosaki University Graduate School of Medicine , Hirosaki,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2026,4,2]]},"reference":[{"key":"2026080515014599800_nlag029-B1","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1038\/nature20413","article-title":"Decoding ALS: from genes to mechanism","volume":"539","author":"Taylor","year":"2016","journal-title":"Nature"},{"key":"2026080515014599800_nlag029-B2","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1093\/jnen\/nlac015","article-title":"Accumulation of nonfibrillar TDP-43 in the rough endoplasmic reticulum is the early-stage pathology in amyotrophic lateral sclerosis","volume":"81","author":"Kon","year":"2022","journal-title":"J Neuropathol Exp Neurol"},{"key":"2026080515014599800_nlag029-B3","doi-asserted-by":"crossref","first-page":"e13215","DOI":"10.1111\/bpa.13215","article-title":"Colocalization of TDP-43 and stress granules at the early stage of TDP-43 aggregation in amyotrophic lateral sclerosis","volume":"34","author":"Mori","year":"2024","journal-title":"Brain Pathol"},{"key":"2026080515014599800_nlag029-B4","doi-asserted-by":"crossref","first-page":"423","DOI":"10.3389\/fncel.2015.00423","article-title":"Alterations in stress granule dynamics driven by TDP-43 and FUS: a link to pathological inclusions in ALS?","volume":"9","author":"Aulas","year":"2015","journal-title":"Front Cell Neurosci"},{"key":"2026080515014599800_nlag029-B5","doi-asserted-by":"crossref","first-page":"668","DOI":"10.1016\/j.tcb.2016.05.004","article-title":"Principles and properties of stress 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