{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,18]],"date-time":"2025-10-18T10:19:31Z","timestamp":1760782771581},"reference-count":41,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2005,7,20]],"date-time":"2005-07-20T00:00:00Z","timestamp":1121817600000},"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 Neuroscience Research"],"published-print":{"date-parts":[[2005,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Amyotrophic lateral sclerosis (ALS) is the most common fatal motor neuron disease. It has been generally accepted that the proapoptotic property of the familial ALS (FALS)\u2010linked mutant <jats:italic>SOD1<\/jats:italic> genes plays an important role in the pathogenesis of some FALS cases. We found here that expression of N19S\u2010SOD1, a novel SOD1 mutant originally found in a sporadic ALS patient, induces lower grade death in NSC34 cells than FALS\u2010linked mutant SOD1. In agreement, intracytoplasmic aggregate formation and SOD1 polymerization are less prominently induced by ectopic expression of N19S\u2010SOD1 than FALS\u2010linked mutant SOD1. We further found that additional cell stresses, such as inhibition of proteasomal activity or up\u2010regulation of intracellular oxidative stress, enhance N19S\u2010SOD1\u2010induced aggregate formation and polymerization of N19S\u2010SOD1. Such analysis of the intracellular polymerization and the ubiquitination of N19S\u2010SOD1 have further suggested that it is recognized as a misfolded protein, like FALS\u2010linked mutant SOD1, whereas wild\u2010type SOD1 is not. Altogether, it is speculated that the N19S mutation of SOD1 in cooperation with associated cell stresses contributes to the onset of ALS as a risk factor. \u00a9 2005 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/jnr.20594","type":"journal-article","created":{"date-parts":[[2005,7,20]],"date-time":"2005-07-20T22:47:25Z","timestamp":1121899645000},"page":"720-729","source":"Crossref","is-referenced-by-count":11,"title":["Expression of N19S\u2010SOD1, an SOD1 mutant found in sporadic amyotrophic lateral sclerosis patients, induceslow\u2010grade motoneuronal toxicity"],"prefix":"10.1002","volume":"81","author":[{"given":"Yuji","family":"Obata","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takako","family":"Niikura","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kohsuke","family":"Kanekura","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuichi","family":"Hashimoto","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masaoki","family":"Kawasumi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoshiko","family":"Kita","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sadakazu","family":"Aiso","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masaaki","family":"Matsuoka","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ikuo","family":"Nishimoto","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2005,7,20]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-4547(19990215)55:4<523::AID-JNR12>3.0.CO;2-Q"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1471-4159.1998.71052041.x"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.281.5384.1851"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.neuro.27.070203.144244"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0361-9230(03)00179-5"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/aja.1001940306"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/jnr.20305"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/35097565"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.10104"},{"key":"e_1_2_6_11_1","first-page":"387","article-title":"Evaluation of the spinal cord neuron \u00d7 neuroblastoma hybrid cell line NSC\u201034 as a model for neurotoxicity testing","volume":"14","author":"Durham HD","year":"1993","journal-title":"Neurotoxicology"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005072-199705000-00008"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1471-4159.1997.69052064.x"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.17-22-08756.1997"},{"key":"e_1_2_6_15_1","first-page":"153","article-title":"Programmed cell death in amyotrophic lateral sclerosis","volume":"111","author":"Guegan C","year":"2003","journal-title":"Neurodegeneration"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.10001"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.101133498"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.10186"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(96)00031-7"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.220417997"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M410508200"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.biocel.2004.05.003"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/114.2.775"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.288.5464.335"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005072-199905000-00005"},{"key":"e_1_2_6_26_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.10605"},{"key":"e_1_2_6_27_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000134608.83927.B1"},{"key":"e_1_2_6_28_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M206559200"},{"key":"e_1_2_6_29_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.9081"},{"key":"e_1_2_6_30_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.240305897"},{"key":"e_1_2_6_31_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.92.7.3024"},{"key":"e_1_2_6_32_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0300-9084(02)00025-1"},{"key":"e_1_2_6_33_1","doi-asserted-by":"publisher","DOI":"10.1038\/362059a0"},{"key":"e_1_2_6_34_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM200105313442207"},{"key":"e_1_2_6_35_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005072-199604000-00011"},{"key":"e_1_2_6_36_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI20682"},{"key":"e_1_2_6_37_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.4253-04.2005"},{"key":"e_1_2_6_38_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1471-4159.2002.01211.x"},{"key":"e_1_2_6_39_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2004.08.003"},{"key":"e_1_2_6_40_1","doi-asserted-by":"publisher","DOI":"10.1038\/4553"},{"key":"e_1_2_6_41_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000123114.04644.CC"},{"key":"e_1_2_6_42_1","doi-asserted-by":"publisher","DOI":"10.1038\/417074a"}],"container-title":["Journal of Neuroscience Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fjnr.20594","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jnr.20594","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,9]],"date-time":"2023-10-09T14:19:18Z","timestamp":1696861158000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/jnr.20594"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2005,7,20]]},"references-count":41,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2005,9]]}},"alternative-id":["10.1002\/jnr.20594"],"URL":"https:\/\/doi.org\/10.1002\/jnr.20594","archive":["Portico"],"relation":{},"ISSN":["0360-4012","1097-4547"],"issn-type":[{"value":"0360-4012","type":"print"},{"value":"1097-4547","type":"electronic"}],"subject":[],"published":{"date-parts":[[2005,7,20]]}}}