{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T18:17:59Z","timestamp":1790187479414,"version":"4.1.0"},"reference-count":22,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2014,10,22]],"date-time":"2014-10-22T00:00:00Z","timestamp":1413936000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100001691","name":"the Japan Society for the Promotion of Science","doi-asserted-by":"crossref","award":["26290017"],"award-info":[{"award-number":["26290017"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2015,2]]},"abstract":"Amyotrophic lateral sclerosis (ALS<\/jats:styled-content>) is a fatal neurodegenerative disease characterized by loss of motor neurons and appearance of skein\u2010like inclusions. The inclusions are composed of trans\u2010activation response (TAR<\/jats:styled-content>) DNA<\/jats:styled-content>\u2010binding protein 43 (TDP<\/jats:styled-content>\u201043), a member of the heterogeneous nuclear ribonucleoprotein (hnRNP<\/jats:styled-content>) family. hnRNPA1<\/jats:styled-content> and hnRNPA2<\/jats:styled-content>\/B<\/jats:styled-content>1 are hnRNPs<\/jats:styled-content> that interact with the C<\/jats:styled-content>\u2010terminus of TDP<\/jats:styled-content>\u201043. Using immunohistochemistry, we investigated the association between TDP<\/jats:styled-content>\u201043 and hnRNPA1<\/jats:styled-content> in ALS<\/jats:styled-content> spinal motor neurons. We examined spinal cords of seven ALS<\/jats:styled-content> cases and six muscular dystrophy cases (used as controls) for the presence of TDP<\/jats:styled-content>\u201043 and hnRNPA1<\/jats:styled-content> protein. In the control cases, hnRNPA1<\/jats:styled-content> immunoreactivity in motor neurons was intense in the nucleus and weak in the cytoplasm where it showed a fine granular appearance. In the ALS<\/jats:styled-content> cases, hnRNPA1<\/jats:styled-content> immunoreactivity in motor neurons was reduced in the nuclei of neurons with skein\u2010like inclusions but was not detected in the skein\u2010like inclusions. The marked loss of hnRNPA1<\/jats:styled-content> in motor neurons with concomitant cytoplasmic aggregation of TDP<\/jats:styled-content>\u201043 may represent a severe disturbance of mRNA<\/jats:styled-content> processing, suggesting a key role in progressive neuronal death in ALS<\/jats:styled-content>.<\/jats:p>","DOI":"10.1111\/neup.12153","type":"journal-article","created":{"date-parts":[[2014,10,22]],"date-time":"2014-10-22T09:02:32Z","timestamp":1413968552000},"page":"37-43","source":"Crossref","is-referenced-by-count":57,"title":["Loss of hnRNPA1<\/scp> in ALS<\/scp> spinal cord motor neurons with TDP<\/scp>\u201043\u2010positive inclusions"],"prefix":"10.1111","volume":"35","author":[{"given":"Hiroyuki","family":"Honda","sequence":"first","affiliation":[{"name":"Department of Neuropathology Neurological Institute Graduate School of Medical Sciences Kyushu University Fukuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hideomi","family":"Hamasaki","sequence":"additional","affiliation":[{"name":"Department of Neuropathology Neurological Institute Graduate School of Medical Sciences Kyushu University Fukuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomihiro","family":"Wakamiya","sequence":"additional","affiliation":[{"name":"Department of Neuropathology Neurological Institute Graduate School of Medical Sciences Kyushu University Fukuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sachiko","family":"Koyama","sequence":"additional","affiliation":[{"name":"Department of Neuropathology Neurological Institute Graduate School of Medical Sciences Kyushu University Fukuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoshi O.","family":"Suzuki","sequence":"additional","affiliation":[{"name":"Department of Neuropathology Neurological Institute Graduate School of Medical Sciences Kyushu University Fukuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naoki","family":"Fujii","sequence":"additional","affiliation":[{"name":"Department of Neurology Neuro\u2010Muscular Center National Omuta Hospital Fukuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toru","family":"Iwaki","sequence":"additional","affiliation":[{"name":"Department of Neuropathology Neurological Institute Graduate School of Medical Sciences Kyushu University Fukuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2014,10,22]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1134108"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbrc.2006.10.093"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(08)70071-1"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1154584"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.132"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.brainres.2012.01.016"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.mcn.2012.12.002"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.mp.4001971"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/emmm.201100995"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature11922"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmb.2005.02.038"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-007-0221-x"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddm276"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20276"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneuroim.2011.02.007"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neulet.2006.03.016"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M505557200"},{"key":"e_1_2_6_19_1","first-page":"96","article-title":"El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. 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