PPIase contributes to EA2-associated defective protein homeostasis of human CaV2.1 channel
Ssu-Ju Fu, Cheng-Tsung Hsiao, Chung-Jiuan Jeng, Chih-Yung Tang · FENS Forum 2024
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ePoster · Neuroscience
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In Neuroscience
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The tottering mouse, which harbors a mutation in the CACNA1A gene encoding a P/Q-type voltage-gated calcium channel, serves as a robust tool in neuroscience research for investigating Epilepsy and Episodic Ataxia Type 2 (EA2). In this study, we characterized the tottering mouse phenotype by leveraging ex vivo (loose-cell) and in vivo (extracellular) electrophysiology recordings of Purkinje cells (PCs) within the cerebellum, along with behavioral tests. Our findings reveal that tottering mice display increased irregularity in action potential (AP) firing of PCs without a change in frequency, bu