CONTEXT

The prevalence of Huntington’s disease (HD) is estimated to 3-7 per 100,000 people in western Europe. HD is caused by a faulty gene on chromosome 4. This gene produces a protein called Huntingtin and leads to a damage of the striatal neurons. The progressive degeneration of these neurons causes gradual physical, mental and emotional changes. At this time, there is no way to stop or to reverse the course of HD. HD is characterized by expansion of CAG codons translated in polyglutamine (polyQ) and causes aggregation of the affected protein. The aim of the invention concerns non toxic compounds capable of treating polyglutamine expansion associated diseases.