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[P100]: Plausible pathogenesis of SCA8 CTG trinucleotide repeats expansion
Author(s) -
Chen I.C.,
Shiau H.C,
Lin H.Y.,
Kao S.H.,
Chen C.M.,
LeeChen G.J.
Publication year - 2006
Publication title -
international journal of developmental neuroscience
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.761
H-Index - 88
eISSN - 1873-474X
pISSN - 0736-5748
DOI - 10.1016/j.ijdevneu.2006.09.162
Subject(s) - chen , china , library science , political science , law , biology , computer science , paleontology
cells transfected with expanded TBP revealed reduced oxidative tolerance upon t-butylhydroperoxide treatment. The reduced oxidative stress tolerance was also observed using lymphoblastoid cell lines from SCA17 patients. Proteomics analysis on transiently transfected 293 and lymphoblastoid cells with TBP expansion further suggests the involvement of proteins assisting folding and metabolism on the disease pathogenesis. The identified abnormalities in neurodegeneration may be used as a “biomarker” for assessing the efficacy of potential therapeutic strategies for SCAs and other polyglutamine diseases.