DISSECTING POLYQ-ATAXIN-1 TRAFFICKING AND NUCLEAR BODY DYNAMICS AS DRIVERS OF TOXICITY
Grant number: 1121907 | Funding period: 2017 - 2019
Ataxias are a large group of neurodegenerative disorders in which balance, motor skills and memory are progressively lost. While mutations in specific proteins do cause certain hereditary ataxias, the mechanisms of their detrimental actions is unclear. Our studies probe the toxic mechanisms of the ataxin-1 protein, focusing on its partners and stress-initiated formation of a toxic hydrogel state. The outcomes will define impacts on cellular protein movement in neurodegeneration more broadly.
Related publications (4)
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Tumor suppressor p53-binding protein 1 (53BP1) is a DNA repair protein essential for the detection, assessment, and resolution of ..
The ataxin-1 interactome reveals direct connection with multiple disrupted nuclear transport pathways
Sunyuan Zhang, Nicholas A Williamson, Lisa Duvick, Alexander Lee, Harry T Orr, Austin Korlin-Downs, Praseuth Yang, Yee-Foong Mok, David A Jans, Marie A Bogoyevitch
The expanded polyglutamine (polyQ) tract form of ataxin-1 drives disease progression in spinocerebellar ataxia type 1 (SCA1). Alth..
Nuclear bodies formed by polyQ-ataxin-1 protein are liquid RNA/protein droplets with tunable dynamics
Sunyuan Zhang, Elizabeth Hinde, Molly Parkyn Schneider, David A Jans, Marie A Bogoyevitch
A mutant form of the ataxin-1 protein with an expanded polyglutamine (polyQ) tract is the underlying cause of the inherited neurod..