What causes selective nerve cell damage?

This chapter describes some of the work which has been undertaken to understand the cause of the selective nerve cell death which occurs in Huntington’s disease (HD). It has long been recognized that the change in the gene results in the protein product doing something which it should not: this is called a gain of function mutation. Despite the identification of the gene nearly 30 years ago a coherent story of the cause of the neurodegeneration has not been established. The chapter describes some of the animal models which have been developed and also the neuronal inclusions or aggregates which develop in cells.

Effect of ethanol as molecular crowding agent on the conformational dynamics of α-synuclein

The functions of many proteins have been directly connected to their conformational changes. The macromolecular crowding environment inside the cell is known to have a significant impact on the equilibria and transition rates between different conformations of the protein. Here we demonstrate the effect of ethanol as crowders on the conformational dynamics of α- synuclein protein, a primary component of the fibrillar neuronal inclusions, and known as Lewy bodies that are diagnostic of Parkinson’s disease. We observed the α-synuclein protein to experience stronger crowding effects with an increase in concentration of ethanol, the crowding agent. The findings that we obtained from this simulation study would serve as valuable guides for expected crowding effects on conformational dynamics of α- synuclein.