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Reversing aberrant phase transitions of RNA-binding proteins connected to ALS and FTD
RNA-binding proteins (RBPs) with prion-like domains (PrLDs) phase transition to functional liquids, which can mature into aberrant hydrogels composed of pathological fibrils that underpin fatal neurodegenerative disorders such as amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Several nuclear RBPs with PrLDs including TDP-43, FUS, hnRNPA1, and hnRNPA2 mislocalize to cytoplasmic inclusions in ALS and FTD and mutations in their PrLDs can accelerate fibrillization and cause disease. Here, I will discuss our latest endeavors to uncover and engineer therapeutic protein disaggregases to reverse these aberrant phase transitions and restore functional RBPs to the nucleus to counter ALS and FTD disease phenotypes.
Date:
18 October 2017, 14:00
Venue:
Dorothy Crowfoot Hodgkin Building, off South Parks Road OX1 3QU
Venue Details:
Seminar Room
Speaker:
Dr James Shorter (Associate Professor, Department of Biochemistry and Biophysics, University of Pennsylvania)
Organising department:
Department of Biochemistry
Organiser:
Dr Tim Nott
Part of:
Seminar
Booking required?:
Not required
Audience:
Members of the University only
Editor:
Carla Bramble
