Neurodegenerative Disorders: Loss of Function Through Gain of Function

A fundamental change is occurring in the understanding of the major neurodegenerative disorders. Protein aggregation is a common emerging theme in diseases as diverse as Alzheimer's, Creutzfeld-Jakob, Parkinson's, and amyotrophic lateral sclerosis. If valid, then a small number of diagnostic and rational therapeutic strategies will emerge over the next few years, based on the common theme of modulation of the production, turnover and deposition of these aggregating proteins. The book provides insights into the cellular and animal models of these diseases, and how the molecular basis of neurodegeneration has become the preferred target of therapy.

The Natural History of Alzheimer’s Disease: Minding the Gaps in Understanding the Mechanisms of Neurodegeneration.- Mechanisms of Motor Neuron Death in ALS.- Pathological Mechanisms in Huntington’s Disease and Other Polyglutamine Expansion Diseases.- Prion Protein Biogenesis: Implications for Neurodegeneration.- The Value of Transgenic Models for the Study of Neurodegenerative Diseases.- Pathogenesis and Mechanism of Cerebral Amyloidosis in APP Transgenic Mice.- Alzheimer’s Disease: Physiological and Pathogenetic Role of the Amyloid Precursor Protein (APP), its A?-Amyloid Domain and Free A?-Amyloid Peptide.- The NEXT Step in Notch Processing and its Relevance to Amyloid Precursor Protein.- The Putative Role of Presenilins in the Transmembrane Domain Cleavage of Amyloid Precursor Protein and Other Integral Membrane Proteins.- ApoE Receptors in the Brain: Novel Signaling Pathways with Potential Relevance for Alzheimer’s Disease.- Homeoprotein Intercellular Transport: Mechanisms, Significance and Applications.- Overexpression of APPL, a Drosophila APP Homologue, Compromises Microtubule Associated Axonal Transport and Promote Synapse Formation.- A Gain of Function of the Huntington’s Disease and Amyotrophic Lateral Sclerosis — Associated Genetic Mutations May Be a Loss of Bioenergetics.