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Publication : Genetic Deletion of Thorase Causes Purkinje Cell Loss and Impaired Motor Coordination Behavior.

First Author  Li C Year  2023
Journal  Cells Volume  12
Issue  16 PubMed ID  37626842
Mgi Jnum  J:340030 Mgi Id  MGI:7524831
Doi  10.3390/cells12162032 Citation  Li C, et al. (2023) Genetic Deletion of Thorase Causes Purkinje Cell Loss and Impaired Motor Coordination Behavior. Cells 12(16)
abstractText  Thorase belongs to the AAA+ ATPase family, which plays a critical role in maintaining cellular homeostasis. Our previous work reported that Thorase was highly expressed in brain tissue, especially in the cerebellum. However, the roles of Thorase in the cerebellum have still not been characterized. In this study, we generated conditional knockout mice (cKO) with Thorase deletion in Purkinje cells. Thorase cKO mice exhibited cerebellar degenerative diseases-like behavior and significant impairment in motor coordination. Thorase deletion resulted in more Purkinje neuron apoptosis, leading to Purkinje cell loss in the cerebellum of Thorase cKO mice. We also found enhanced expression of the inflammatory protein ASC, IL-1beta, IL-6 and TNF-alpha in the Thorase cKO cerebellum, which contributed to the pathogenesis of cerebellar degenerative disease. Our findings provide a better understanding of the role of Thorase in the cerebellum, which is a theoretical basis for Thorase as a therapeutic drug target for neurodegenerative diseases.
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