spinocerebellar ataxias as diseases of Purkinje cell dysfunction rather than Purkinje cell loss

JP Kapfhammer, E Shimobayashi - Frontiers in Molecular …, 2023 - frontiersin.org
Spinocerebellar ataxias (SCAs) are a group of hereditary neurodegenerative diseases
mostly affecting cerebellar Purkinje cells caused by a wide variety of different mutations. One …

Endosomal dysfunction contributes to cerebellar deficits in spinocerebellar ataxia type 6

AA Cook, TCS Leung, M Rice, M Nachman… - Elife, 2023 - elifesciences.org
Abstract Spinocerebellar ataxia type 6 (SCA6) is a rare disease that is characterized by
cerebellar dysfunction. Patients have progressive motor coordination impairment, and …

[HTML][HTML] Phenotypical, genotypical and pathological characterization of the moonwalker mouse, a model of ataxia

G Sekerková, S Kilic, YH Cheng, N Fredrick… - Neurobiology of …, 2024 - Elsevier
We performed a comprehensive study of the morphological, functional, and genetic features
of moonwalker (MWK) mice, a mouse model of spinocerebellar ataxia caused by a gain of …

Commissural and monosynaptic inputs to medial vestibular nucleus GABAergic neurons in mice

D Kong, L Kong, C Liu, Q Wu, J Wang, C Dai - Frontiers in Neurology, 2024 - frontiersin.org
Objective MVN GABAergic neurons is involved in the rebalance of commissural system
contributing to alleviating acute peripheral vestibular dysfunction syndrome. This study aims …