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SUMMARY:BMI SEMINAR // Magdalini Polymenidou - Untangling ALS and FTLD pat
 hogenesis: from molecular structures to ageing neural systems
DTSTART:20200205T121500
DTEND:20200205T131500
DTSTAMP:20260929T061108Z
UID:c4a8a164a33cd3ea1324e4566e605d19fd86259b9544c9c91a0bf073
CATEGORIES:Conferences - Seminars
DESCRIPTION:Magdalini Polymenidou\, Molecular pathogenesis of neurodegener
 ation\, Department of Quantitative Biomedicine\, University of Zurich\, CH
 \nAmyotrophic lateral sclerosis (ALS) and frontotemporal dementia FTD are 
 fatal and incurable neurodegenerative diseases\, characterized by accumula
 tion of pathologic forms of RNA-binding proteins\, most frequently TDP-43 
 and FUS. The molecular triggers of TDP-43 and aggregation\, the basis of d
 isease heterogeneity and the mechanisms of neurotoxicity remain elusive. T
 o address these important questions\, we study the formation and propertie
 s of the physiological and pathological states of these proteins in patien
 t samples and disease cellular and animal models. Combining structural pro
 tein analysis with cellular systems we uncovered a novel and unexpected me
 chanism that counteracts pathologic aggregation of TDP-43 (1). We recently
  discovered that ALS and FTD heterogeneity is associated with alternate pa
 thological TDP-43 conformations\, reminiscent of prion strains (2). Moreov
 er\, we have described a trigger for the initial cytoplasmic mislocalizati
 on of FUS\, likely occurring in sporadic FTD-FUS patients (3). The implica
 tions of all this work and its potential for gaining mechanistic insights 
 on disease will be discussed.\n 
LOCATION:SV 1717 https://plan.epfl.ch/?room==SV%201717
STATUS:CONFIRMED
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