Article
An arginine/lysine-rich motif is crucial for VCP/p97-mediated modulation of ataxin-3 fibrillogenesis.
The EMBO journal - 5 Apr 2006
Boeddrich Annett, Gaumer Sébastien, Haacke Annette, Tzvetkov Nikolay, Albrecht Mario, Evert Bernd O, Müller Eva C, Lurz Rudi, Breuer Peter, Schugardt Nancy, Plassmann Stephanie, Xu Kexiang, Warrick John M, Suopanki Jaana, Wüllner Ullrich, Frank Ronald, Hartl Ulrich F, Bonini Nancy M, Wanker Erich E
Abstract excerpt
Arginine/lysine-rich motifs typically function as targeting signals for the translocation of proteins to the nucleus. Here, we demonstrate that such a motif consisting of four basic amino acids in the polyglutamine protein ataxin-3 (Atx-3) serves as a recognition site for the interaction with the molecular chaperone VCP. Through this interaction, VCP modulates the fibrillogenesis of pathogenic forms of Atx-3 in a...
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