Animal models of neurodegenerative dementing disorders other than Alzheimer's disease

Abstract

Neurodegenerative dementias other than Alzheimer's disease (AD) are complex disorders with diverse causes. Recently, there has been tremendous progress in the pathobiology and genetics of several of these diseases, some of which are associated with pathological alterations in tau and α-synuclein. These findings have led to the generation of murine and invertebrate transgenic tau and α-synuclein animal models of these disorders to facilitate elucidation of mechanisms underlying these disorders and for discovery of novel therapeutic interventions to treat them. The focus of this review will be to provide an overview of diseases that have been modeled by these transgenic animals and to highlight the findings of these studies. Overall, transgenic models have supported the notion that tau and α-synuclein play central roles in the pathobiology of many non-AD dementias and have provided important insights into the mechanisms that lead to these disorders thereby setting the stage for identifying novel targets for pharmacological interventions as well as proof of concept studies of compounds with therapeutic potential in patients.