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SUMMARY:Special LMNN Seminar - Self-propagating huntingtin aggregates and 
 their potential role in Huntington’s disease
DTSTART:20180321T140000
DTEND:20180321T150000
DTSTAMP:20260930T195023Z
UID:fcad49f5b2726ee470fca597a5edee8f42d7ebba7ec4f1e3894b188e
CATEGORIES:Conferences - Seminars
DESCRIPTION:Erich E. Wanker\, Max Delbrueck Center for Molecular Medicine 
 (MDC) in the Helmholtz Association\, Berlin\, GERMANY\nSelf-propagation o
 f amyloidogenic protein aggregates may drive the progression of neurodegen
 erative diseases including Alzheimer’s disease (AD)\, Parkinson’s dise
 ase (PD) and Huntington’s disease (HD). We recently developed a cell-fre
 e\, FRET-based mutant huntingtin (mHTT) aggregate seeding (FRASE) biosenso
 r assay that enables the detection and quantification of mHTT seeding acti
 vity (HSA) in complex biosamples from HD patients and disease models. Appl
 ication of FRASE assays revealed HSA in crude brain homogenates of presymp
 tomatic HD transgenic mice and its progressive increase with development o
 f the phenotype\, indicating that HSA quantitatively tracks disease progre
 ssion. Biochemical investigations of mouse brain homogenates demonstrated 
 that HSA is detectable in soluble protein fractions that contain small mHT
 T fibrils. Furthermore\, studies in an inducible Drosophila fly model reve
 aled that the short-time production of seeding-competent mHTTex1 aggregate
 s in fly brains is sufficient to reduce their lifespan\, suggesting that m
 utant HTTex1 seeds are highly toxic structures. Together\, our results sup
 port the hypothesis that self-propagating mHTT seeds play a critical role 
 in HD.\n\n 
LOCATION:AI 1153 https://plan.epfl.ch/?room==AI%201153
STATUS:CONFIRMED
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