{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,17]],"date-time":"2025-10-17T13:41:46Z","timestamp":1760708506375},"reference-count":31,"publisher":"Public Library of Science (PLoS)","issue":"3","license":[{"start":{"date-parts":[[2013,3,7]],"date-time":"2013-03-07T00:00:00Z","timestamp":1362614400000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"DOI":"10.1371\/journal.pone.0050121","type":"journal-article","created":{"date-parts":[[2013,3,7]],"date-time":"2013-03-07T16:58:38Z","timestamp":1362675518000},"page":"e50121","update-policy":"http:\/\/dx.doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":11,"title":["Cytoplasmic Location of \u03b11A Voltage-Gated Calcium Channel C-Terminal Fragment (Cav2.1-CTF) Aggregate Is Sufficient to Cause Cell Death"],"prefix":"10.1371","volume":"8","author":[{"given":"Makoto","family":"Takahashi","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masato","family":"Obayashi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Taro","family":"Ishiguro","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nozomu","family":"Sato","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yusuke","family":"Niimi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kokoro","family":"Ozaki","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kaoru","family":"Mogushi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yasen","family":"Mahmut","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroshi","family":"Tanaka","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fuminori","family":"Tsuruta","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ricardo","family":"Dolmetsch","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mitsunori","family":"Yamada","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hitoshi","family":"Takahashi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takeo","family":"Kato","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Osamu","family":"Mori","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoshinobu","family":"Eishi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hidehiro","family":"Mizusawa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kinya","family":"Ishikawa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"340","published-online":{"date-parts":[[2013,3,7]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"575","DOI":"10.1146\/annurev.neuro.29.051605.113042","article-title":"Trinucleotide repeat disorders","volume":"30","author":"HT Orr","year":"2007","journal-title":"Annu Rev Neurosci"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"R115","DOI":"10.1093\/hmg\/ddm213","article-title":"Polyglutamine diseases: emerging concepts in pathogenesis and therapy","volume":"16 Spec No. 2","author":"J Shao","year":"2007","journal-title":"Hum Mol Genet"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1038\/ng0197-62","article-title":"Autosomal dominant cerebellar ataxia (SCA6) associated with small polyglutamine expansions in the alpha 1A-voltage-dependent calcium channel","volume":"15","author":"O Zhuchenko","year":"1997","journal-title":"Nat Genet"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"336","DOI":"10.1086\/514867","article-title":"Japanese families with autosomal dominant pure cerebellar ataxia map to chromosome 19p13.1-p13.2 and are strongly associated with mild CAG expansions in the spinocerebellar ataxia type 6 gene in chromosome 19p13.1","volume":"61","author":"K Ishikawa","year":"1997","journal-title":"Am J Hum Genet"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"933","DOI":"10.1002\/ana.410420616","article-title":"Spinocerebellar ataxia type 6: gaze-evoked and vertical nystagmus, Purkinje cell degeneration, and variable age of onset","volume":"42","author":"CM Gomez","year":"1997","journal-title":"Ann Neurol"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"1185","DOI":"10.1093\/hmg\/8.7.1185","article-title":"Abundant expression and cytoplasmic aggregations of [alpha]1A voltage-dependent calcium channel protein associated with neurodegeneration in spinocerebellar ataxia type 6","volume":"8","author":"K Ishikawa","year":"1999","journal-title":"Hum Mol Genet"},{"key":"ref7","doi-asserted-by":"crossref","first-page":"RC14","DOI":"10.1523\/JNEUROSCI.19-12-j0004.1999","article-title":"Direct alteration of the P\/Q-type Ca2+ channel property by polyglutamine expansion in spinocerebellar ataxia 6","volume":"19","author":"Z Matsuyama","year":"1999","journal-title":"J Neurosci"},{"key":"ref8","doi-asserted-by":"crossref","first-page":"10893","DOI":"10.1074\/jbc.275.15.10893","article-title":"Spinocerebellar ataxia type 6 mutation alters P-type calcium channel function","volume":"275","author":"S Toru","year":"2000","journal-title":"J Biol Chem"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"6394","DOI":"10.1523\/JNEUROSCI.20-17-06394.2000","article-title":"The polyglutamine expansion in spinocerebellar ataxia type 6 causes a beta subunit-specific enhanced activation of P\/Q-type calcium channels in Xenopus oocytes","volume":"20","author":"S Restituito","year":"2000","journal-title":"J Neurosci"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"9185","DOI":"10.1523\/JNEUROSCI.21-23-09185.2001","article-title":"Increased expression of alpha 1A Ca2+ channel currents arising from expanded trinucleotide repeats in spinocerebellar ataxia type 6","volume":"21","author":"ES Piedras-Renteria","year":"2001","journal-title":"J Neurosci"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"261","DOI":"10.1016\/j.mcn.2006.11.006","article-title":"Properties of human Cav2.1 channel with a spinocerebellar ataxia type 6 mutation expressed in Purkinje cells","volume":"34","author":"H Saegusa","year":"2007","journal-title":"Mol Cell Neurosci"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"11987","DOI":"10.1073\/pnas.0804350105","article-title":"Spinocerebellar ataxia type 6 knockin mice develop a progressive neuronal dysfunction with age-dependent accumulation of mutant CaV2.1 channels","volume":"105","author":"K Watase","year":"2008","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"1753","DOI":"10.1212\/WNL.56.12.1753","article-title":"Cytoplasmic and nuclear polyglutamine aggregates in SCA6 Purkinje cells","volume":"56","author":"K Ishikawa","year":"2001","journal-title":"Neurology"},{"key":"ref14","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1016\/S0022-510X(98)00009-4","article-title":"SCA6 mutation analysis in a large cohort of the Japanese patients with late-onset pure cerebellar ataxia","volume":"156","author":"I Yabe","year":"1998","journal-title":"J Neurol Sci"},{"key":"ref15","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1007\/s10038-004-0142-7","article-title":"A clinical and genetic study in a large cohort of patients with spinocerebellar ataxia type 6","volume":"49","author":"H Takahashi","year":"2004","journal-title":"J Hum Genet"},{"key":"ref16","doi-asserted-by":"crossref","first-page":"1990","DOI":"10.1126\/science.277.5334.1990","article-title":"Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain","volume":"277","author":"M DiFiglia","year":"1997","journal-title":"Science"},{"key":"ref17","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1016\/S0896-6273(00)80943-5","article-title":"Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3","volume":"19","author":"HL Paulson","year":"1997","journal-title":"Neuron"},{"key":"ref18","doi-asserted-by":"crossref","first-page":"447","DOI":"10.1007\/s00401-009-0630-0","article-title":"The carboxy-terminal fragment of alpha(1A) calcium channel preferentially aggregates in the cytoplasm of human spinocerebellar ataxia type 6 Purkinje cells","volume":"119","author":"T Ishiguro","year":"2010","journal-title":"Acta Neuropathol"},{"key":"ref19","doi-asserted-by":"crossref","first-page":"1587","DOI":"10.1093\/hmg\/ddl080","article-title":"C-termini of P\/Q-type Ca2+ channel alpha1A subunits translocate to nuclei and promote polyglutamine-mediated toxicity","volume":"15","author":"HB Kordasiewicz","year":"2006","journal-title":"Hum Mol Genet"},{"key":"ref20","doi-asserted-by":"crossref","first-page":"1253","DOI":"10.1111\/j.1365-2443.2009.01348.x","article-title":"Deficit of heat shock transcription factor 1-heat shock 70 kDa protein 1A axis determines the cell death vulnerability in a model of spinocerebellar ataxia type 6","volume":"14","author":"L Li","year":"2009","journal-title":"Genes Cells"},{"key":"ref21","doi-asserted-by":"crossref","first-page":"1088","DOI":"10.1111\/j.1600-0854.2008.00743.x","article-title":"Toxicity and endocytosis of spinocerebellar ataxia type 6 polyglutamine domains: role of myosin IIb","volume":"9","author":"B Marqueze-Pouey","year":"2008","journal-title":"Traffic"},{"key":"ref22","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1038\/79139","article-title":"Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription","volume":"26","author":"T Shimohata","year":"2000","journal-title":"Nat Genet"},{"key":"ref23","doi-asserted-by":"crossref","first-page":"2197","DOI":"10.1093\/hmg\/9.14.2197","article-title":"CREB-binding protein sequestration by expanded polyglutamine","volume":"9","author":"A McCampbell","year":"2000","journal-title":"Hum Mol Genet"},{"key":"ref24","doi-asserted-by":"crossref","first-page":"2423","DOI":"10.1126\/science.1056784","article-title":"Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity","volume":"291","author":"FC Nucifora Jr","year":"2001","journal-title":"Science"},{"key":"ref25","doi-asserted-by":"crossref","first-page":"178","DOI":"10.1016\/j.neuron.2010.01.008","article-title":"Early increase in extrasynaptic NMDA receptor signaling and expression contributes to phenotype onset in Huntington&apos;s disease mice","volume":"65","author":"AJ Milnerwood","year":"2010","journal-title":"Neuron"},{"key":"ref26","article-title":"Reduced brain-derived neurotrophic factor (BDNF) mRNA expression and presence of BDNF-immunoreactive granules in the spinocerebellar ataxia type 6 (SCA6) cerebellum","author":"M Takahashi","year":"2012","journal-title":"Neuropathology"},{"key":"ref27","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1093\/hmg\/ddg002","article-title":"Cell death triggered by polyglutamine-expanded huntingtin in a neuronal cell line is associated with degradation of CREB-binding protein","volume":"12","author":"H Jiang","year":"2003","journal-title":"Hum Mol Genet"},{"key":"ref28","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1007\/BF02742437","article-title":"The early cellular pathology of Huntington&apos;s disease","volume":"20","author":"XJ Li","year":"1999","journal-title":"Mol Neurobiol"},{"key":"ref29","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1038\/ncb1677","article-title":"Intra-axonal translation and retrograde trafficking of CREB promotes neuronal survival","volume":"10","author":"LJ Cox","year":"2008","journal-title":"Nat Cell Biol"},{"key":"ref30","doi-asserted-by":"crossref","first-page":"5208","DOI":"10.1021\/bi002732+","article-title":"Nuclear import of Creb and AP-1 transcription factors requires importin-beta 1 and Ran but is independent of importin-alpha","volume":"40","author":"JK Forwood","year":"2001","journal-title":"Biochemistry"},{"key":"ref31","doi-asserted-by":"crossref","first-page":"17870","DOI":"10.1074\/jbc.M602632200","article-title":"Functional repression of cAMP response element in 6-hydroxydopamine-treated neuronal cells","volume":"281","author":"EM Chalovich","year":"2006","journal-title":"J Biol Chem"}],"container-title":["PLoS ONE"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pone.0050121","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,7,10]],"date-time":"2019-07-10T12:32:01Z","timestamp":1562761921000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0050121"}},"subtitle":[],"editor":[{"given":"J. David","family":"Spafford","sequence":"first","affiliation":[],"role":[{"role":"editor","vocabulary":"crossref"}]}],"short-title":[],"issued":{"date-parts":[[2013,3,7]]},"references-count":31,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2013,3,7]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pone.0050121","relation":{},"ISSN":["1932-6203"],"issn-type":[{"value":"1932-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,3,7]]}}}