ArticleBrain research bulletin2020
The abnormal firing of Purkinje cells in the knockin mouse model of DYT1 dystonia.
Article in Brain research bulletin, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed, 21 citations in OpenAlex.
- Validation of a torsinA cerebellar knockdown model of DYT1 dystonia.Dystonia (Lausanne, Switzerland) · 2026Article
- Subtle changes in Purkinje cell firing in Purkinje cell-specificDystonia (Lausanne, Switzerland) · 2025Article
- Cerebellar contributions to dystonia: unraveling the role of Purkinje cells and cerebellar nuclei.Dystonia (Lausanne, Switzerland) · 2025Article
- Sex-specific alterations of Purkinje cell firing inDystonia (Lausanne, Switzerland) · 2025Article
- Physiological Recordings of the Cerebellum in Movement Disorders.Cerebellum (London, England) · 2023Review
- Cell-specific Dyt1 ∆GAG knock-in to basal ganglia and cerebellum reveal differential effects on motor behavior and sensorimotor network function.Experimental neurology · 2023Article
- Cerebellar Dysfunction as a Source of Dystonic Phenotypes in Mice.Cerebellum (London, England) · 2023Article
- Electromyography as a Method for Distinguishing Dystonia in Mice.Advances in neurobiology · 2023Article
- DYT-Frontiers in neuroscience · 2023Review
- Further Studies on the Role of BTBD9 in the Cerebellum, Sleep-like Behaviors and the Restless Legs Syndrome.Neuroscience · 2022Article
- Hyperactivity of Purkinje cell and motor deficits in C9orf72 knockout mice.Molecular and cellular neurosciences · 2022Article
- Electrophysiological characterization of the striatal cholinergic interneurons inDystonia (Lausanne, Switzerland) · 2022Article
- Cerebellum Involvement in Dystonia During Associative Motor Learning: Insights From a Data-Driven Spiking Network Model.Frontiers in systems neuroscience · 2022Article
- Quantification of Behavioral Deficits in Developing Mice With Dystonic Behaviors.Dystonia (Lausanne, Switzerland) · 2022Article
- Cerebellar Direct Current Stimulation (ctDCS) in the Treatment of Huntington's Disease: A Pilot Study and a Short Review of the Literature.Frontiers in neurology · 2020Article
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Authors and funding
7 authors at 1 institution in 1 country.
Funding
Abstract
DYT1 dystonia is an inherited movement disorder caused by a heterozygous trinucleotide (GAG) deletion in DYT1/TOR1A, coding for torsinA. Growing evidence suggests that the cerebellum plays a role in the pathogenesis of dystonia. Brain imaging of both DYT1 dystonia patients and animal models show abnormal activity in the cerebellum. The cerebellum-specific knockdown of torsinA in adult mice leads to dystonia-like behavior. Dyt1 ΔGAG heterozygous knock-in mouse model exhibits impaired corticostriatal long-term depression, abnormal muscle co-contraction, and motor deficits. We and others previously reported altered dendritic structures in Purkinje cells in Dyt1 knock-in mouse models. However, whether there are any electrophysiological alterations of the Purkinje cells in Dyt1 knock-in mice is not known. We used the patch-clamp recording in brain slices and in acutely dissociated Purkinje cells to identify specific alterations of Purkinje cells firing. We found abnormal firing of non-tonic type of Purkinje cells in the Dyt1 knock-in mice. Furthermore, the large-conductance calcium-activated potassium (BK) current and the BK channel protein levels were significantly increased in the Dyt1 knock-in mice. Our results support a role of the cerebellum in the pathogenesis of DYT1 dystonia. Manipulating the Purkinje cell firing and cerebellar output may show great promise for treating DYT1 dystonia.
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