Troubles neurologiques

Polyglutamine Diseases-Understanding the Mechanism of Pathogenesis

Abstract

Sonali Sengupta and Saurabh Bhattacharya

Protein misfolding has been implicated in a large number of diseases, which are now grouped under the name of Protein conformational disorders (PCDs). Few examples of diseases that fall in this group are Alzheimer’s disease, Parkinson’s disease and Huntington’s disease. All these disorders are characterized by sets of protein that misfold and aggregate in specific tissues. In order to identify and develop possible routes of therapeutic strategies, scientists have discovered several modifiers for these fatal diseases. These modifiers, primarily identified using models systems, include heat shock proteins, components of UPS pathway and autophagy, transcription factors, detoxifying enzymes, several RNA binding proteins, and RNA species, among other examples. These reviews will focus primarily on cellular processes that are affected in Polyglutamine disorders.