Biologic models of neurodegenerative disorders

Abstract

Publisher Summary In human neurodegenerative disorders, genetic mutations that are identified in familiar forms of disease have served as a starting point for recapitulating the underlying pathological processes in biologic models. The central rationale supporting the development of biologic models is that they enable researchers to apply powerful experimental tools to understand the fundamental biology of neurodegenerative disease. The chapter describes how the mutant forms of genes that are associated with pathological phenotypes have been cloned into transgenic mouse, fly, and worm models. The chapter elucidates that utilizing a mutant gene that has only been shown to be associated with a small fraction of disease cases may yield an incomplete or misleading model. Despite these potential pitfalls, most models of neurodegenerative disease recapitulate some aspects of the disease and enhance our understanding of the disorder. The chapter further explains that the bulk of the evidence supporting the use of these models currently favors disturbances in basic cellular processes that are tightly conserved between vertebrate and invertebrate species.