ArticleThe Journal of neuroscience : the official journal of the Society for Neuroscience2014
Abnormal high-frequency burst firing of cerebellar neurons in rapid-onset dystonia-parkinsonism.
Article in The Journal of neuroscience : the official journal of the Society for Neuroscience, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 72 papers.
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Who cites it
72 citing papers in PubMed, 119 citations in OpenAlex.
- A prefrontal cortex-piriform cortex circuit mediates diminished interest.Molecular psychiatry · 2026Article
- Cerebellar Electrical Activity as a Marker for Predicting Brain Health and Disease: A Review.Brain sciences · 2026Review
- Disinhibition of cerebellar output by loss of restless legs syndrome-associated gene MEIS1.Current biology : CB · 2026Article
- Cerebellar motor and non-motor contributions to dystonia pathophysiology and treatment.Dystonia (Lausanne, Switzerland) · 2026Article
- Processing reliant on granule cells is essential for motor learning but dispensable for social preference and numerous other cerebellar-dependent behaviors.Nature communications · 2025Article
- Aberrant outputs of glutamatergic neurons in deep cerebellar nuclei mediate dystonic movements.Science advances · 2025Article
- Thalamic deep brain stimulation improves movement in a cerebellar model of lesion-based status dystonicus.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2025Article
- Review
- Cerebellar contributions to dystonia: unraveling the role of Purkinje cells and cerebellar nuclei.Dystonia (Lausanne, Switzerland) · 2025Article
- Article
- Targeting DBS to the centrolateral thalamic nucleus improves movement in a lesion-based model of acquired cerebellar dystonia in mice.bioRxiv : the preprint server for biology · 2024Article
- Striatal parvalbumin interneurons are activated in a mouse model of cerebellar dystonia.Disease models & mechanisms · 2024Article
- State-Dependent Blockade of Dorsal Root Ganglion Voltage-Gated NaInternational journal of molecular sciences · 2024Article
- Cerebellar Dysfunction as a Source of Dystonic Phenotypes in Mice.Cerebellum (London, England) · 2023Article
- Cortical neuronal hyperexcitability and synaptic changes in SGCE mutation-positive myoclonus dystonia.Brain : a journal of neurology · 2023Article
- Function and dysfunction of the dystonia network: an exploration of neural circuits that underlie the acquired and isolated dystonias.Dystonia (Lausanne, Switzerland) · 2023Article
- Cerebellar dysfunction in rodent models with dystonia, tremor, and ataxia.Dystonia (Lausanne, Switzerland) · 2023Article
- A disynaptic basal ganglia connection to the inferior olive: potential for basal ganglia influence on cerebellar learning.Frontiers in systems neuroscience · 2023Article
- Article
- Cerebellar glutamatergic system impacts spontaneous motor recovery by regulating Gria1 expression.NPJ Regenerative medicine · 2022Article
12 more citing papers are in PubMed but not listed here.
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4 authors at 1 institution in 1 country.
Funding
Abstract
Loss-of-function mutations in the α3 isoform of the Na(+)/K(+) ATPase (sodium pump) are responsible for rapid-onset dystonia parkinsonism (DYT12). Recently, a pharmacological model of DYT12 was generated implicating both the cerebellum and basal ganglia in the disorder. Notably, partially blocking sodium pumps in the cerebellum was necessary and sufficient for induction of dystonia. Thus, a key question that remains is how partially blocking sodium pumps in the cerebellum induces dystonia. In vivo recordings from dystonic mice revealed abnormal high-frequency bursting activity in neurons of the deep cerebellar nuclei (DCN), which comprise the bulk of cerebellar output. In the same mice, Purkinje cells, which provide strong inhibitory drive to DCN cells, also fired in a similarly erratic manner. In vitro studies demonstrated that Purkinje cells are highly sensitive to sodium pump dysfunction that alters the intrinsic pacemaking of these neurons, resulting in erratic burst firing similar to that identified in vivo. This abnormal firing abates when sodium pump function is restored and dystonia caused by partial block of sodium pumps can be similarly alleviated. These findings suggest that persistent high-frequency burst firing of cerebellar neurons caused by sodium pump dysfunction underlies dystonia in this model of DYT12.
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