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Abnormalities in signal transduction of Purkinje cells in spinocerebellar ataxias: a review

WU Qiong^1,2,3, HUANG Qing-Yun^1,2,3, TAN Guo-He^1,2,3,*

¹Center for Translational Medicine, Guangxi Medical University, Nanning 530021, China；²Key Laboratory of Longevity and Aging-related Diseases of Chinese Ministry of Education, Nanning 530021, China；³Guangxi Key Laboratory of Brain Research, School of Basic Medical Sciences, Guangxi Medical University, Nanning 530021, China

Abstract

Spinocerebellar ataxias (SCAs) are a group of autosomal dominant neurodegenerative diseases that have been currently identified with numerous subtypes exhibiting genetic heterogeneity and clinical variability. Purkinje neuronal degeneration and cerebellar atrophy are common pathological features among most SCA subtypes. The physiological functions of Purkinje cells are regulated by multiple factors, and their dysfunction in signal transduction may lead to abnormal cerebellar motor control. This review summarizes the abnormalities in voltage-gated ionic channels, intracellular calcium signaling, and glutamate signaling transduction of Purkinje cells in SCAs, aiming to provide a theoretical basis for further understanding the common pathogenesis of SCAs and developing specific treatments.

Key words: spinocerebellar ataxias; Purkinje cells; signal transduction; ion channels

Received: 　　Accepted:

Corresponding author: 谭国鹤　　E-mail: tanguohe@gxmu.edu.cn

DOI: 10.13294/j.aps.2023.0043

Citing This Article：

WU Qiong, HUANG Qing-Yun, TAN Guo-He. Abnormalities in signal transduction of Purkinje cells in spinocerebellar ataxias: a review. Acta Physiol Sin 2024; 76 (1): 77-88 (in Chinese with English abstract).