Article
Cellular toxicity of polyglutamine expansion proteins: mechanism of transcription factor deactivation.
Molecular cell - 2 Jul 2004
Schaffar Gregor, Breuer Peter, Boteva Raina, Behrends Christian, Tzvetkov Nikolay, Strippel Nadine, Sakahira Hideki, Siegers Katja, Hayer-Hartl Manajit, Hartl F Ulrich
Abstract excerpt
The expression of polyglutamine-expanded mutant proteins in Huntington's disease and other neurodegenerative disorders is associated with the formation of intraneural inclusions. These aggregates could potentially cause cellular toxicity by sequestering essential proteins possessing normal polyQ repeats, including the transcription factors TBP and CBP. We show, in vitro and in cells, that monomers or small...
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