Professor, Department of Pharmacology & Toxicology, University of Arizona
"RNA Binding Proteins and Neurodegenerative Diseases"
Host: Andrew Paek
RNA binding proteins and dysregulation of RNA metabolism are an emerging theme in neurodegenerative diseases. We will discuss the RNA binding protein Fused in Sarcoma (FUS) whose mutations are implicated in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Our laboratory has characterized the wild-type and disease-causing mutants of FUS using a variety of approaches including proteomics, biochemistry, cell biology and genetic models. Our recent studies show that the ALS/FTD mutations of FUS suppress protein translation and dysregulate mRNA decay. We will also discuss the post-translational modifications, particularly acetylation, of FUS that regulate its subcellular localization, RNA binding, and formation of protein-RNA condensates.