In double-transgenic mouse models non-expanded ataxin-3 not suppresses polyglutamine neurodegeneration in the SCA3 disease – in vivo evidence

Central questions in polyQ-induced neurodegeneration are the influence of the function of the non-expanded and the disease protein, the formation of aggregates, and the modulation by quality control mechanisms (e.g. unfolded protein response, ERAD or ubiquitin proteasome pathway). To answer the question of the interaction of a non-expanded allele with an expanded polyQ stretch in vivo we generated double-transgenic (dt) mouse models for the neurodegenerative disease spinocerebellar ataxia type 3 (SCA3). SCA3, also known as Machado-Joseph disease (MJD), is the most common type of spinocerebellar ataxias in Europe…