ArticleBrain pathology (Zurich, Switzerland)2006
Spinocerebellar ataxia type 3 (SCA3): thalamic neurodegeneration occurs independently from thalamic ataxin-3 immunopositive neuronal intranuclear inclusions.
Article in Brain pathology (Zurich, Switzerland), 2006. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers.
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Who cites it
29 citing papers in PubMed, 67 citations in OpenAlex.
- Allosteric Modulation of Pathological Ataxin-3 Aggregation: A Path to Spinocerebellar Ataxia Type-3 Therapies.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2026Article
- Gray Matter Asymmetry Alterations in Patients With Spinocerebellar Ataxia Type 3: A Voxel-Based Morphometric Comparison Study.CNS neuroscience & therapeutics · 2024Article
- Neuro-respiratory pathology in spinocerebellar ataxia.Journal of the neurological sciences · 2022Review
- A survey of protein interactions and posttranslational modifications that influence the polyglutamine diseases.Frontiers in molecular neuroscience · 2022Review
- Quantitative susceptibility mapping of basal ganglia iron is associated with cognitive and motor functions that distinguish spinocerebellar ataxia type 6 and type 3.Frontiers in neuroscience · 2022Article
- Mutant Ataxin-3-Containing Aggregates (MATAGGs) in Spinocerebellar Ataxia Type 3: Dynamics of the Disorder.Molecular neurobiology · 2021Review
- Supratentorial and Infratentorial Lesions in Spinocerebellar Ataxia Type 3.Frontiers in neurology · 2020Article
- From Pathogenesis to Novel Therapeutics for Spinocerebellar Ataxia Type 3: Evading Potholes on the Way to Translation.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2019Review
- On the distribution of intranuclear and cytoplasmic aggregates in the brainstem of patients with spinocerebellar ataxia type 2 and 3.Brain pathology (Zurich, Switzerland) · 2017Article
- Precision medicine in spinocerebellar ataxias: treatment based on common mechanisms of disease.Annals of translational medicine · 2016Review
- Change in the cortical complexity of spinocerebellar ataxia type 3 appears earlier than clinical symptoms.PloS one · 2015Article
- Limited Effect of Chronic Valproic Acid Treatment in a Mouse Model of Machado-Joseph Disease.PloS one · 2015Article
- Parkinsonism in spinocerebellar ataxia.BioMed research international · 2015Review
- The role for alterations in neuronal activity in the pathogenesis of polyglutamine repeat disorders.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2014Review
- Ataxin-3 protein and RNA toxicity in spinocerebellar ataxia type 3: current insights and emerging therapeutic strategies.Molecular neurobiology · 2014Review
- Stable polyglutamine dimers can contain β-hairpins with interdigitated side chains-but not α-helices, β-nanotubes, β-pseudohelices, or steric zippers.Biophysical journal · 2014Article
- Chronic treatment with 17-DMAG improves balance and coordination in a new mouse model of Machado-Joseph disease.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2014Article
- Trinucleotide repeats: a structural perspective.Frontiers in neurology · 2013Article
- Cognitive deficits in Machado-Joseph disease correlate with hypoperfusion of visual system areas.Cerebellum (London, England) · 2012Article
- Pathoanatomy of cerebellar degeneration in spinocerebellar ataxia type 2 (SCA2) and type 3 (SCA3).Cerebellum (London, England) · 2012Article
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14 authors at 1 institution in 1 country.
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No grant is acknowledged in the PubMed record.
Abstract
In the last years progress has been made regarding the involvement of the thalamus during the course of the currently known polyglutamine diseases. Although recent studies have shown that the thalamus consistently undergoes neurodegeneration in Huntington's disease (HD) and spinocerebellar ataxia type 2 (SCA2) it is still unclear whether it is also a consistent target of the pathological process of spinocerebellar ataxia type 3 (SCA3). Accordingly we studied the thalamic pathoanatomy and distribution pattern of ataxin-3 immunopositive neuronal intranuclear inclusions (NI) in nine clinically diagnosed and genetically confirmed SCA3 patients and carried out a detailed statistical analysis of our findings. During our pathoanatomical study we disclosed (i) a consistent degeneration of the ventral anterior, ventral lateral and reticular thalamic nuclei; (ii) a degeneration of the ventral posterior lateral nucleus and inferior and lateral subnuclei of the pulvinar in the majority of these SCA3 patients; and (iii) a degeneration of the ventral posterior medial and lateral posterior thalamic nuclei, the lateral geniculate body and some of the limbic thalamic nuclei in some of them. Upon immunocytochemical analysis we detected NI in all of the thalamic nuclei of all of our SCA3 patients. According to our statistical analysis (i) thalamic neurodegeneration and the occurrence of ataxin-3 immunopositive thalamic NI was not associated with the individual length of the CAG-repeats in the mutated SCA3 allele, the patients age at disease onset and the duration of SCA3 and (ii) thalamic neurodegeneration was not correlated with the occurrence of ataxin-3 immunopositive thalamic NI. This lack of correlation may suggest that ataxin-3 immunopositive NI are not immediately decisive for the fate of affected nerve cells but rather represent unspecific and pathognomonic morphological markers of SCA3.
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