{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,1]],"date-time":"2025-10-01T16:30:28Z","timestamp":1759336228234},"reference-count":63,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2007,12,18]],"date-time":"2007-12-18T00:00:00Z","timestamp":1197936000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Comparative Neurology"],"published-print":{"date-parts":[[2008,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p><jats:italic>Dystonia musculorum<\/jats:italic> (<jats:italic>dt<\/jats:italic>) is a mutant mouse with hereditary neuropathy where the dysfunction is mainly found in the dorsal root ganglia (DRG) neurons but not in the spinal motor neurons. However, the accumulation of intermediate filament (IF) proteins in the swelling axons of spinal motor neurons could be found in <jats:italic>dt\/dt<\/jats:italic> mice. In order to understand the pathological role of neuronal IFs in the swelling axons of spinal motor neurons from <jats:italic>dt\/dt<\/jats:italic> mice, we extensively examined the distribution of neuronal IF proteins. By immunofluorescence staining, our results indicated that \u03b1\u2010internexin was a major component in the swelling axon and showed abnormal translocation in the nuclei of spinal motor neurons in <jats:italic>dt\/dt<\/jats:italic> mice. This abnormal translocation of \u03b1\u2010internexin in the nuclei of spinal motor neurons was also confirmed by Western blotting and immunoelectron microscopy. Instead of the 10\u2010nm filamentous structure, a diffuse immunopositive pattern of \u03b1\u2010internexin was observed in the nucleus of spinal motor neurons in <jats:italic>dt\/dt<\/jats:italic> mutants. We further examined the cell death of spinal motor neurons by TUNEL assay, and no TUNEL\u2010positive cells could be identified from spinal motor neurons in <jats:italic>dt\/dt<\/jats:italic> mice. From these observations we suggest that abnormal accumulation of neuronal IFs in the swelling axons and abnormal translocation of \u03b1\u2010internexin in the nuclei of the spinal motor neurons from <jats:italic>dt\/dt<\/jats:italic> mice may not directly cause cell death of the spinal motor neurons. J. Comp. Neurol. 507:1053\u20131064, 2008. \u00a9 2007 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/cne.21606","type":"journal-article","created":{"date-parts":[[2007,12,18]],"date-time":"2007-12-18T17:47:05Z","timestamp":1198000025000},"page":"1053-1064","source":"Crossref","is-referenced-by-count":11,"title":["Abnormal cellular translocation of \u03b1\u2010internexin in spinal motor neurons of <i>Dystonia musculorum<\/i> mice"],"prefix":"10.1002","volume":"507","author":[{"given":"Kuang\u2010Wen","family":"Tseng","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yat\u2010Pang","family":"Chau","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mei\u2010Fang","family":"Yang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kuo\u2010Shyan","family":"Lu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chung\u2010Liang","family":"Chien","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2007,12,21]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0305-7372(03)00081-1"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1056-8719(97)00033-6"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.147.3.531"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.20-14-05321.2000"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng0795-301"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000139809.16817.DD"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9440(10)63773-X"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-4695(199603)29:3<304::AID-NEU3>3.0.CO;2-D"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-4547(19981101)54:3<353::AID-JNR6>3.0.CO;2-6"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/jnr.20506"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.122.6.1323"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/0896-6273(89)90189-X"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.pharmtox.45.120403.095902"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(93)90158-M"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1471-4159.2003.01861.x"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1006\/dbio.1997.8567"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/87.2.367"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005072-198807000-00004"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awh278"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0074-7696(08)62018-5"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1002\/j.1460-2075.1990.tb08169.x"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1002\/cne.903420202"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/0165-3806(90)90049-5"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.281.5381.1309"},{"key":"e_1_2_6_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(95)90333-X"},{"key":"e_1_2_6_27_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00054014"},{"key":"e_1_2_6_28_1","doi-asserted-by":"publisher","DOI":"10.1124\/mol.51.4.542"},{"key":"e_1_2_6_29_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/95.3.529"},{"key":"e_1_2_6_30_1","doi-asserted-by":"crossref","first-page":"2735","DOI":"10.1523\/JNEUROSCI.10-08-02735.1990","article-title":"Alpha\u2010internexin, a novel neuronal intermediate filament protein, precedes the low molecular weight neurofilament protein (NF\u2010L) in the developing rat brain","volume":"10","author":"Kaplan MP","year":"1990","journal-title":"J Neurosci"},{"key":"e_1_2_6_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/0248-4900(92)90193-5"},{"key":"e_1_2_6_32_1","doi-asserted-by":"publisher","DOI":"10.1038\/335435a0"},{"key":"e_1_2_6_33_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.physiol.60.1.619"},{"key":"e_1_2_6_34_1","doi-asserted-by":"publisher","DOI":"10.1016\/0165-3806(82)90192-4"},{"key":"e_1_2_6_35_1","doi-asserted-by":"publisher","DOI":"10.1002\/neu.10270"},{"key":"e_1_2_6_36_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.ne.19.030196.001155"},{"issue":"2","key":"e_1_2_6_37_1","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1093\/brain\/112.2.521","article-title":"Cytoskeletal abnormalities in motor neuron disease. 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