| http://purl.uniprot.org/citations/18502140 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/18502140 | http://www.w3.org/2000/01/rdf-schema#comment | "In the present study, we prepared a SCA3 animal model by generating transgenic mice expressing polyglutamine-expanded ataxin-3-Q79. Ataxin-3-Q79 was expressed in brain areas implicated in SCA3 neurodegeneration, including cerebellum, pontine nucleus and substantia nigra. Ataxin-3-Q79 transgenic mice displayed motor dysfunction with an onset age of 5-6 months, and neurological symptoms deteriorated in the following months. A prominent neuronal loss was not found in the cerebellum of 10 to 11-month-old ataxin-3-Q79 mice displaying pronounced ataxic symptoms, suggesting that instead of neuronal demise, ataxin-3-Q79 causes neuronal dysfunction of the cerebellum and resulting ataxia. To test the involvement of transcriptional dysregulation in ataxin-3-Q79-induced cerebellar malfunction, microarray analysis and real-time RT-PCR assays were performed to identify altered cerebellar mRNA expressions of ataxin-3-Q79 mice. Compared to non-transgenic mice or mice expressing wild-type ataxin-3-Q22, 10 to 11-month-old ataxin-3-Q79 mice exhibited downregulated mRNA expressions of proteins involved in glutamatergic neurotransmission, intracellular calcium signaling/mobilization or MAP kinase pathways, GABA(A/B) receptor subunits, heat shock proteins and transcription factor regulating neuronal survival and differentiation. Upregulated expressions of Bax, cyclin D1 and CDK5-p39, which may mediate neuronal death, were also observed in ataxin-3-Q79 transgenic mice. The involvement of transcriptional abnormality in initiating the pathological process of SCA3 was indicated by the finding that 4 to 5-month-old ataxin-3-Q79 mice, which did not display neurological phenotype, exhibited downregulated mRNA levels of genes involved in glutamatergic signaling and signal transduction. Our study suggests that polyglutamine-expanded ataxin-3 causes cerebellar dysfunction and ataxia by disrupting the normal pattern of gene transcriptions."xsd:string |
| http://purl.uniprot.org/citations/18502140 | http://purl.org/dc/terms/identifier | "doi:10.1016/j.nbd.2008.03.011"xsd:string |
| http://purl.uniprot.org/citations/18502140 | http://purl.uniprot.org/core/author | "Chen Y.L."xsd:string |
| http://purl.uniprot.org/citations/18502140 | http://purl.uniprot.org/core/author | "Wang H.L."xsd:string |
| http://purl.uniprot.org/citations/18502140 | http://purl.uniprot.org/core/author | "Chen S.Y."xsd:string |
| http://purl.uniprot.org/citations/18502140 | http://purl.uniprot.org/core/author | "Ouyang P."xsd:string |
| http://purl.uniprot.org/citations/18502140 | http://purl.uniprot.org/core/author | "Yeh T.H."xsd:string |
| http://purl.uniprot.org/citations/18502140 | http://purl.uniprot.org/core/author | "Chou A.H."xsd:string |
| http://purl.uniprot.org/citations/18502140 | http://purl.uniprot.org/core/date | "2008"xsd:gYear |
| http://purl.uniprot.org/citations/18502140 | http://purl.uniprot.org/core/name | "Neurobiol Dis"xsd:string |
| http://purl.uniprot.org/citations/18502140 | http://purl.uniprot.org/core/pages | "89-101"xsd:string |
| http://purl.uniprot.org/citations/18502140 | http://purl.uniprot.org/core/title | "Polyglutamine-expanded ataxin-3 causes cerebellar dysfunction of SCA3 transgenic mice by inducing transcriptional dysregulation."xsd:string |
| http://purl.uniprot.org/citations/18502140 | http://purl.uniprot.org/core/volume | "31"xsd:string |
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