ASIP Outstanding Investigator Award Lecture. New approaches to the pathology and genetics of neurodegeneration.

The pathologies of major neurodegenerative diseases including Parkinson disease and Alzheimer disease have been well known for decades. More recently, advances in molecular genetics have suggested important mechanistic links between the pathology of these disorders and pathogenesis of neuronal dysfunction and death. Numerous animal models have been produced based on the new information emerging from human genetic studies. As a complement to traditional mouse models, a number of investigators have modeled neurodegenerative diseases in simple model organisms ranging from yeast to Drosophila. These simple genetic models often display remarkable pathological similarities to their cognate human disorders, and genetic and biochemical studies have yielded important insights into the pathogenesis of the human disorders. Use of these tractable simple models may become even more important as large amounts of genetic data emerge from genome-wide association studies in Alzheimer disease, Parkinson disease, and other neurodegenerative disorders.