Molecular basis for neurodegeneration in Ataxia telangiectasia

There is increasing evidence that links together various common neurodegenerative diseases, such as Parkinson’s and Alzheimer’s diseases. Finding common themes in the pathophysiology of such disorders is increasingly important. This text addresses such a common theme—the proteasome.

Since the discovery of the proteasome some 20 years ago, significant strides have been made in our understanding of proteasome biology and our understanding of the role the proteasome plays in a wide variety of biochemical events. Taken together, these studies have confirmed an important role for the proteasome in the fields of cell biology, oncology, immunology, gerontology, and neuroscience. The focus of this book is to provide an in depth analysis and provocative discussion of what...

Looking at each specific neurological disorder in turn, the book outlines the role of metals in human biology, in particular in the brain and explores tools for testing potential therapeutic strategies. It concludes with an overview of the potential of both chelation and antioxidant therapy and outlines some perspectives for the future.

For the last four decades, the study of the functioning and pathological alterations of the Nervous System has been intensified in search for therapeutic alternatives against neurodegenerative disorders in humans. Since their first descriptions in the 19th and 20th centuries, sufficient clinical and experimental findings contributed to describe the origin of some of the most common and frequent neurological diseases. Derived from this multidisciplinary research performed along decades, now we know that a great number of factors trigger nerve tissue damage and cell death, and they include the environmental exposure to toxicants, inheritance patterns, the individual susceptibility to endogenous or exogenous neurotoxins, ageing processes, traumatic injury, drug...