Aggregation of the DNA/RNA-binding protein FUS (FUsed in Sarcoma) in the cytoplasm of nerve cells is characteristic of two devastating and incurable neurodegenerative disorders. Amyotrophic lateral sclerosis (ALS) is a motor neuron disease, which leads to progressive muscle weakness and ultimately to lethal paralysis, while frontotemporal dementia (FTD), the second most common form of dementia (after Alzheimer’s) in those under 65. GSN/MCN member Dorothee Dormann and her group (including GSN PhD Student Mario Hofweber) are studying the molecular mechanisms that underlie both of these conditions. In a new study, they report that the aggregation of FUS observed in ALS and FTD is attributable to two distinct but functionally related molecular mechanisms. Their findings are reported in the leading journal Cell.
Source: LMU News