The role of heat shock proteins in PolyQ disorders

PhD ceremony: Ms. M.P. Zijlstra, 13.15 uur, Academiegebouw, Broerstraat 5, Groningen

Title: The role of heat shock proteins in PolyQ disorders

Promotor(s): prof. H.H. Kampinga, prof. O.C.M. Sibon

Faculty: Medical Sciences

Some neurodegenerative diseases are the result of aggregation of damaged proteins in cells of the central nervous system. Huntington’s disease is a well-known example. The focus of this thesis was to study the role of heat shock proteins in protein quality control routes in relation to the progression of neurodegenerative diseases. Various types of heat shock proteins were found with different effects on protein aggregation. A heat shock protein with activity on autophagy, the bulk degradation system of the cell, was identified as the strongest suppressor of protein aggregation in the neurodegenerative diseases under study. Experiments with heat shock proteins in model systems indicated possibilities for reducing progression in neurodegenerative diseases.