• Anna A Cook, Eviatar Fields, and Alanna J Watt.
• Department of Biology, McGill University, Montreal, Canada.
• Neuroscience. 2021 May 10; 462: 247-261.

AbstractThe cerebellum is a brain structure that is highly interconnected with other brain regions. There are many contributing factors to cerebellar-related brain disease, such as altered afferent input, local connectivity, and/or cerebellar output. Purkinje cells (PC) are the principle cells of the cerebellar cortex, and fire intrinsically; that is, they fire spontaneous action potentials at high frequencies. This review paper focuses on PC intrinsic firing activity, which is altered in multiple neurological diseases, including ataxia, Huntington Disease (HD) and autism spectrum disorder (ASD). Notably, there are several cases where interventions that restore or rescue PC intrinsic activity also improve impaired behavior in these mouse models of disease. These findings suggest that rescuing PC firing deficits themselves may be sufficient to improve impairment in cerebellar-related behavior in disease. We propose that restoring PC intrinsic firing represents a good target for drug development that might be of therapeutic use for several disorders.Copyright © 2020 The Author(s). Published by Elsevier Ltd.. All rights reserved.

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