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  3. Σχολή Επιστημών Υγείας
  4. Τμήμα Ιατρικής
  5. Άρθρα σε επιστημονικά περιοδικά ( Ανοικτά) - ΙΑΤ
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Please use this identifier to cite or link to this item: https://olympias.lib.uoi.gr/jspui/handle/123456789/21285
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dc.contributor.authorAdachi, H.en
dc.contributor.authorKatsuno, M.en
dc.contributor.authorMinamiyama, M.en
dc.contributor.authorSang, C.en
dc.contributor.authorPagoulatos, G.en
dc.contributor.authorAngelidis, C.en
dc.contributor.authorKusakabe, M.en
dc.contributor.authorYoshiki, A.en
dc.contributor.authorKobayashi, Y.en
dc.contributor.authorDoyu, M.en
dc.contributor.authorSobue, G.en
dc.date.accessioned2015-11-24T19:14:19Z-
dc.date.available2015-11-24T19:14:19Z-
dc.identifier.issn1529-2401-
dc.identifier.urihttps://olympias.lib.uoi.gr/jspui/handle/123456789/21285-
dc.rightsDefault Licence-
dc.subjectAnimalsen
dc.subjectBlotting, Westernen
dc.subjectCell Nucleus/*metabolism/pathologyen
dc.subjectCrosses, Geneticen
dc.subjectDisease Models, Animalen
dc.subjectDisease Progressionen
dc.subjectGene Expressionen
dc.subjectHSP70 Heat-Shock Proteins/*biosynthesis/geneticsen
dc.subjectHumansen
dc.subjectImmunohistochemistryen
dc.subjectMacromolecular Substancesen
dc.subjectMaleen
dc.subjectMiceen
dc.subjectMice, Transgenicen
dc.subjectMolecular Chaperones/*biosynthesis/geneticsen
dc.subjectMotor Activity/geneticsen
dc.subjectMuscular Atrophy, Spinal/genetics/pathology/*physiopathologyen
dc.subjectMutationen
dc.subjectPhenotypeen
dc.subjectReceptors, Androgen/genetics/*metabolismen
dc.subjectTrinucleotide Repeat Expansion/geneticsen
dc.titleHeat shock protein 70 chaperone overexpression ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model by reducing nuclear-localized mutant androgen receptor proteinen
heal.typejournalArticle-
heal.type.enJournal articleen
heal.type.elΆρθρο Περιοδικούel
heal.identifier.secondaryhttp://www.ncbi.nlm.nih.gov/pubmed/12657679-
heal.languageen-
heal.accesscampus-
heal.recordProviderΠανεπιστήμιο Ιωαννίνων. Σχολή Επιστημών Υγείας. Τμήμα Ιατρικήςel
heal.publicationDate2003-
heal.abstractSpinal and bulbar muscular atrophy (SBMA) is an inherited motor neuron disease caused by the expansion of the polyglutamine (polyQ) tract within the androgen receptor (AR). The nuclear inclusions consisting of the mutant AR protein are characteristic and combine with many components of ubiquitin-proteasome and molecular chaperone pathways, raising the possibility that misfolding and altered degradation of mutant AR may be involved in the pathogenesis. We have reported that the overexpression of heat shock protein (HSP) chaperones reduces mutant AR aggregation and cell death in a neuronal cell model (Kobayashi et al., 2000). To determine whether increasing the expression level of chaperone improves the phenotype in a mouse model, we cross-bred SBMA transgenic mice with mice overexpressing the inducible form of human HSP70. We demonstrated that high expression of HSP70 markedly ameliorated the motor function of the SBMA model mice. In double-transgenic mice, the nuclear-localized mutant AR protein, particularly that of the large complex form, was significantly reduced. Monomeric mutant AR was also reduced in amount by HSP70 overexpression, suggesting the enhanced degradation of mutant AR. These findings suggest that HSP70 overexpression ameliorates SBMA phenotypes in mice by reducing nuclear-localized mutant AR, probably caused by enhanced mutant AR degradation. Our study may provide the basis for the development of an HSP70-related therapy for SBMA and other polyQ diseases.en
heal.journalNameJ Neuroscien
heal.journalTypepeer-reviewed-
heal.fullTextAvailabilityTRUE-
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