Article
Identification of human proteins that modify misfolding and proteotoxicity of pathogenic ataxin-1.
PLoS genetics - 1 Jan 2012
Petrakis Spyros, Raskó Tamás, Russ Jenny, Friedrich Ralf P, Stroedicke Martin, Riechers Sean-Patrick, Muehlenberg Katja, Möller Angeli, Reinhardt Anita, Vinayagam Arunachalam, Schaefer Martin H, Boutros Michael, Tricoire Hervé, Andrade-Navarro Miguel A, Wanker Erich E
Abstract excerpt
Proteins with long, pathogenic polyglutamine (polyQ) sequences have an enhanced propensity to spontaneously misfold and self-assemble into insoluble protein aggregates. Here, we have identified 21 human proteins that influence polyQ-induced ataxin-1 misfolding and proteotoxicity in cell model systems. By analyzing the protein sequences of these modifiers, we discovered a recurrent presence of coiled-coil (CC)...
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