2019
DOI: 10.1002/kjm2.12106
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Treadmill training increases the motor activity and neuron survival of the cerebellum in a mouse model of spinocerebellar ataxia type 1

Abstract: Spinocerebellar ataxia (SCA) type 1 (SCA1) is a rare autosomal dominant disorder that is characterized by worsening of disordered coordination, ataxia of the trunk, and other neurological symptoms. Physical activity improves both mobility and the daily living activities of patients with SCA. Intervention with daily regular treadmill exercise may slow the deterioration of cerebellar neurons in SCA1. Therefore, the signal changes and performance of cerebellar neurons after exercise in SCA1 was investigated in th… Show more

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Cited by 12 publications
(9 citation statements)
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“…In the anterior vermis characterized by three thin stripes of AldoC-positive Purkinje cells (25), these stripes are unreliably present in ATXN1[82Q] mice. We also compared AldoC expression with an additional marker of Purkinje cell pathology, i.e., nuclear inclusions that represent a mid-late hallmark in ATXN1[82Q] Purkinje cells (43). To label nuclear inclusions, we stained for p62/SQSTM1 (p62 hereafter), an autophagy adaptor protein that is present at high levels in many neuronal inclusions, including intranuclear inclusions of SCA1 patients (44).…”
Section: Aldoc Expression Is Disrupted In Atxn1[82q] Micementioning
confidence: 99%
“…In the anterior vermis characterized by three thin stripes of AldoC-positive Purkinje cells (25), these stripes are unreliably present in ATXN1[82Q] mice. We also compared AldoC expression with an additional marker of Purkinje cell pathology, i.e., nuclear inclusions that represent a mid-late hallmark in ATXN1[82Q] Purkinje cells (43). To label nuclear inclusions, we stained for p62/SQSTM1 (p62 hereafter), an autophagy adaptor protein that is present at high levels in many neuronal inclusions, including intranuclear inclusions of SCA1 patients (44).…”
Section: Aldoc Expression Is Disrupted In Atxn1[82q] Micementioning
confidence: 99%
“…In this scenario, identifying mutations related to several hereditary ataxias has led to developing a growing number of valuable animal models (Manto and Marmolino, 2009). As an example, the study of NP in models of spinocerebellar ataxia (SCA) type 1 (SCA1) concluded that external factors (like regular treadmill exercise) changed vital regulatory pathways in the structural and functional plasticity of the neuron, play a critical role in the viable support of SCA1 mice cerebellar neurons (Chuang et al, 2019). More important, (due to overlapping mechanisms across different NDDs) such mutations could have common molecular pathways by genetically conserved NP mechanisms, amenable to be potentially repaired using the proper molecular tools.…”
Section: Other Neurodegenerative Diseasesmentioning
confidence: 99%
“…Exercise has been shown to rescue deficits in an SCA1 mouse model 28,29 and in ataxic Snf2h-null mice 30 , although BDNF has not been identified as the mechanism underlying its therapeutic action in these models. We wondered whether exercise, perhaps via elevation of cerebellar BDNF levels, would rescue motor behavioural deficits in SCA6 84Q/84Q mice.…”
Section: Resultsmentioning
confidence: 99%