PROJECT 2:

Host:

Max Planck Institute for Biophysical Chemistry

 

PI:
Dirk Goerlic

Former ESR:
Metin Aksu

Ataxin-3 is normally a cytoplasmic protein. In its pathological polyQ-extended forms, however, it shows intranuclear inclusions - a location that appears critical for pathogenesis. We are currently investigating how polyQ-extended forms of ataxin-3 reach the nucleus. While we so far failed in detecting any direct binding to nuclear transport receptors, we observed a direct interaction with FG domains that form the permeability barrier of nuclear pore complexes (NPCs). This could indicate that ataxin-3 is able to traverse NPCs in a receptor-independent manner and that its steady-state localisation is primarily determined by cytoplasmic or intranuclear retention.