Other Medicine and Health Sciences Commons^™

The Role Of Polyglutamine Aggregate Cytotoxicity In Huntington’S Disease, Wen Yang

Doctoral Dissertations

Polyglutamine diseases refer to a group of neuro-degenerative diseases including Huntington's disease , that share the same mutational basis: expansion of a CAG repeat coding sequence that encodes for a polyglutamine repeat in the respective proteins. Neuronal intranuclear inclusions (NII) formed by the aggregation of the expanded polyglutamine proteins is a hallmark of polyglutamine diseases. However, it is still under debate whether the polyglutamine aggregates are toxic to the cells, and if so, which aggregated forms are the toxic species. While important data has been provided by the existing transfected cell and transgenic animal models, technical aspects have limited our …