Publisher's Synopsis
This book focuses on the discovery of a common genetic basis for a group of inherited neurological disorders including Huntington's Disease, spino-bulbar muscular atrophy and a series of hereditary ataxias. It now seems likely that these disorders are triggered by expanded trinucleotide repeats resulting in polyglutamine sequences in the specific proteins produced by the genes. This shared molecular background and other similarities, have led to the development of theoretical models for the pathogenesis of these diseases. It is now clear that the mechanisms involved are likely to be of more general relevance, outside of this particular group of disorders, with implications for other neurodegenerative processes such as those involved in Alzheimer's, Parkinson's and Prion diseases. The book starts with a review of the animal models of Huntington's Disease, before moving on to examine the issue of polyglutamine toxicity and the biochemistry of the Huntingtin protein.;The latter part of the book describes how these discoveries have brought a new unity to the molecular pathology of neurodegenerative diseases, and describes other disease groups thought to be related to the presence of proteins with expanded glutamine repeats. This book is an edited and updated compilation evolving from a Royal Society discussion meeting.
