Protein Misfolding Disorders

Event Details

Protein Misfolding Disorders

Time: July 18, 2011 to July 30, 2011
Location: San Quirico d'Orcia, Siena
Street: Palazzo Chigi Zondadari - Piazza Chigi 2
City/Town: San Quirico d'Orcia, Siena, Italy
Website or Map: http://www.nsas.it/protein-mi…
Event Type: course
Organized By: Neuroscience School of Advanced Studies
Latest Activity: Apr 19, 2011

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Event Description

Those of us who attended medical school in the 1980s may remember that the pathological aggregation of proteins, and formation of amyloid, was thought to be a rare and curious phenomenon affecting a small number of select proteins. This view has been thoroughly revised: we have now learned that most, if not all, proteins can give rise to highly ordered aggregates that propagate similarly to crystals. Even more importantly, the spectrum of diseases caused by the inappropriate aggregation of proteins has increased dramatically.
It has now become clear that many of the most prevalent neurodegenerative diseases, including Alzheimer's, Parkinson's, and many others, progress in ways that are similar to those of the classic systemic amyloidosis. Although prion diseases were originally thought to propagate through instructional changes of the cellular prion protein, it is now clear that nucleation, fibril elongation, and fragmentation also underlies prion infectivity. Conversely, aggregates in Alzheimer’s, tauopathies and synucleinopathies spread within the brain similarly to prions and have been termed "prionoids".
The Course will examine the chemistry and biophysics governing amyloid nucleation and propagation, its cellular biology, the mechanism by which protein aggregates damage the brain, and the resulting diagnostic and therapeutic challenges. The explicit goal of the Course is to foster communication between scientists from disparate disciplines (from nuclear magnetic resonance spectroscopy to cell biology, transgenic mouse models, and clinical science) in order to germinate innovative steps towards deeper understanding, sensitive diagnosis, and effective treatments of these devastating diseases.
The Neuroscience School of Advanced Studies is the home of residential, intensive Courses, where leading investigators from around the world spend two full weeks discussing upcoming research challenges with a small, highly selected number of participants in the unique atmosphere of the fortifies medieval village of San Quirico d'Orcia, all within the most idyllic Tuscan countryside.

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