{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,8]],"date-time":"2026-09-08T12:30:13Z","timestamp":1788870613413,"version":"build-2803163510"},"reference-count":41,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2013,1,15]],"date-time":"2013-01-15T00:00:00Z","timestamp":1358208000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"German Genome Network NGFNplus"},{"DOI":"10.13039\/501100003493","name":"Hertie Foundation","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100003493","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100005224","name":"German Center for Neurodegenerative Diseases","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100005224","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal of Neurochemistry"],"published-print":{"date-parts":[[2013,4]]},"abstract":"Abstract<\/jats:title>\n \n DJ<\/jats:styled-content>\n \u20101 is a ubiquitous protein regulating cellular viability. Recessive mutations in the\n PARK<\/jats:styled-content>\n 7\/\n DJ<\/jats:styled-content>\n \u20101 gene are linked to Parkinson's disease (\n PD<\/jats:styled-content>\n ). Although the most dramatic L166P point mutation practically eliminates\n DJ<\/jats:styled-content>\n \u20101 protein and function, the effects of other\n PD<\/jats:styled-content>\n \u2010linked mutations are subtler. Here, we investigated two recently described\n PD<\/jats:styled-content>\n \u2010associated\n DJ<\/jats:styled-content>\n \u20101 point mutations, the A179T substitution and the P158\u0394 in\u2010frame deletion. [A179T]\n DJ<\/jats:styled-content>\n \u20101 protein was as stable as wild\u2010type [wt]\n DJ<\/jats:styled-content>\n \u20101, but the P158\u0394 mutant protein was less stable. In accord with the notion that dimer formation is essential for\n DJ<\/jats:styled-content>\n \u20101 protein stability, [P158\u0394]\n DJ<\/jats:styled-content>\n \u20101 was impaired in dimer formation. Similar to our previous findings for [M26I]\n DJ<\/jats:styled-content>\n \u20101, [P158\u0394]\n DJ<\/jats:styled-content>\n \u20101 bound aberrantly to apoptosis signal\u2010regulating kinase 1. Thus, the\n PD<\/jats:styled-content>\n \u2010associated P158\u0394 mutation destabilizes\n DJ<\/jats:styled-content>\n \u20101 protein and function. As there is also evidence for an involvement of\n DJ<\/jats:styled-content>\n \u20101 in multiple system atrophy, a\n PD<\/jats:styled-content>\n \u2010related \u03b1\u2010synucleinopathy characterized by oligodendroglial cytoplasmic inclusions, we studied an oligodendroglial cell line stably expressing \u03b1\u2010synuclein. \u03b1\u2010Synuclein aggregate dependent microtubule retraction upon co\u2010transfection with tubulin polymerization\u2010promoting protein p25\u03b1 was ameliorated by [wt]\n DJ<\/jats:styled-content>\n \u20101. In contrast,\n DJ<\/jats:styled-content>\n \u20101 mutants including P158\u0394 failed to protect in this system, where we found evidence of apoptosis signal\u2010regulating kinase 1 (\n ASK<\/jats:styled-content>\n 1) involvement. In conclusion, the P158\u0394 point mutation may contribute to neurodegeneration by protein destabilization and hence loss of\n DJ<\/jats:styled-content>\n \u20101 function.\n <\/jats:p>","DOI":"10.1111\/jnc.12126","type":"journal-article","created":{"date-parts":[[2012,12,14]],"date-time":"2012-12-14T07:02:53Z","timestamp":1355468573000},"page":"314-327","source":"Crossref","is-referenced-by-count":17,"title":["Loss of\n DJ<\/scp>\n \u20101 protein stability and cytoprotective function by\n P<\/scp>\n arkinson's disease\u2010associated proline\u2010158 deletion"],"prefix":"10.1111","volume":"125","author":[{"given":"Emmy H.","family":"Rannikko","sequence":"first","affiliation":[{"name":"Laboratory of Functional Neurogenetics Department of Neurodegeneration Hertie Institute for Clinical Brain Research Faculty of Medicine University of T\u00fcbingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Louise Buur","family":"Vesterager","sequence":"additional","affiliation":[{"name":"Department of Biomedicine Faculty of Health Sciences University of Aarhus Denmark"},{"name":"H. Lundbeck A\/S Department of Neurodegeneration Valby Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jafar H. A.","family":"Shaik","sequence":"additional","affiliation":[{"name":"Department of Biomedicine Faculty of Health Sciences University of Aarhus Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephanie S.","family":"Weber","sequence":"additional","affiliation":[{"name":"Laboratory of Functional Neurogenetics Department of Neurodegeneration Hertie Institute for Clinical Brain Research Faculty of Medicine University of T\u00fcbingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena M.","family":"Cornejo Castro","sequence":"additional","affiliation":[{"name":"Laboratory of Functional Neurogenetics Department of Neurodegeneration Hertie Institute for Clinical Brain Research Faculty of Medicine University of T\u00fcbingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karina","family":"Fog","sequence":"additional","affiliation":[{"name":"H. Lundbeck A\/S Department of Neurodegeneration Valby Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Poul H.","family":"Jensen","sequence":"additional","affiliation":[{"name":"Department of Biomedicine Faculty of Health Sciences University of Aarhus Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philipp J.","family":"Kahle","sequence":"additional","affiliation":[{"name":"Laboratory of Functional Neurogenetics Department of Neurodegeneration Hertie Institute for Clinical Brain Research Faculty of Medicine University of T\u00fcbingen Germany"},{"name":"German Center for Neurodegenerative Diseases T\u00fcbingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,1,15]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.10675"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0709379104"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0001884"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1077209"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0402959101"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.281.5384.1860"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M509079200"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.00227-07"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M309204200"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M609821200"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neures.2010.04.002"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0409635102"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.freeradbiomed.2009.08.003"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbrc.2004.03.110"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2004.06.006"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M809671200"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M410409200"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.2008.05333.x"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddg304"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.22287"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0601891103"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M304272200"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1002\/jcb.22530"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.2003.02265.x"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1091\/mbc.E07-05-0491"},{"key":"e_1_2_7_27_1","doi-asserted-by":"publisher","DOI":"10.1091\/mbc.E09-03-0211"},{"key":"e_1_2_7_28_1","doi-asserted-by":"publisher","DOI":"10.1006\/bbrc.1997.6132"},{"key":"e_1_2_7_29_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-004-0834-2"},{"key":"e_1_2_7_30_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M506771200"},{"key":"e_1_2_7_31_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M311017200"},{"key":"e_1_2_7_32_1","doi-asserted-by":"publisher","DOI":"10.1002\/jnr.22477"},{"key":"e_1_2_7_33_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-4547(19960715)45:2<161::AID-JNR8>3.0.CO;2-8"},{"key":"e_1_2_7_34_1","doi-asserted-by":"publisher","DOI":"10.1089\/ars.2009.2832"},{"key":"e_1_2_7_35_1","doi-asserted-by":"publisher","DOI":"10.1093\/emboj\/17.9.2596"},{"key":"e_1_2_7_36_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pbio.0020362"},{"key":"e_1_2_7_37_1","doi-asserted-by":"publisher","DOI":"10.2353\/ajpath.2007.070201"},{"key":"e_1_2_7_38_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.pharmtox.48.113006.094606"},{"key":"e_1_2_7_39_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M304221200"},{"key":"e_1_2_7_40_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M806902200"},{"key":"e_1_2_7_41_1","doi-asserted-by":"publisher","DOI":"10.1089\/ars.2010.3481"},{"key":"e_1_2_7_42_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M507124200"}],"container-title":["Journal 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