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Cerebellar Biochemistry/Pharmacology

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Trials for Cerebellar Ataxias

Part of the book series: Contemporary Clinical Neuroscience ((CCNE))

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Abstract

Cerebellar Purkinje cells (PCs) are localized in the cerebellar cortex and are characterized by highly developed dendrites, which receive different inputs from the parallel fibers of cerebellar granule cells, climbing fibers of inferior olive neurons in the medulla, and other cerebellar interneurons. PCs are the sole output neurons of the cerebellar cortex and are crucial for cerebellar functions. PC degeneration and morphological changes in PC dendrites are frequently observed in postmortem patients and mouse models of various cerebellar ataxias. Therefore, the factors that regulate the survival and morphology of PCs may be associated with the pathogenesis and progression of cerebellar ataxia. In this chapter, I summarize the interactions between PCs and other cerebellar cells that affect the survival and morphology of cerebellar PCs. Furthermore, numerous studies have revealed that intracellular protein degradation systems contribute to the maintenance of protein homeostasis and are essential for neuronal survival and function retention. Therefore, I also outline the role of protein degradation systems in the regulation of the survival and function of cerebellar neurons. Lastly, I briefly describe the endogenous modulators that affect the survival and morphology of cerebellar PCs.

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Acknowledgment

I would like to thank Editage (www.editage.com) for English language editing.

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Correspondence to Takahiro Seki .

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© 2023 The Author(s), under exclusive license to Springer Nature Switzerland AG

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Seki, T. (2023). Cerebellar Biochemistry/Pharmacology. In: Soong, Bw., Manto, M., Brice, A., Pulst, S.M. (eds) Trials for Cerebellar Ataxias. Contemporary Clinical Neuroscience. Springer, Cham. https://doi.org/10.1007/978-3-031-24345-5_3

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