ArticleThe Journal of neuroscience : the official journal of the Society for Neuroscience2013
A novel transgenic rat model for spinocerebellar ataxia type 17 recapitulates neuropathological changes and supplies in vivo imaging biomarkers.
Article in The Journal of neuroscience : the official journal of the Society for Neuroscience, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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Who cites it
25 citing papers in PubMed, 39 citations in OpenAlex.
- OBSERVE: guidelines for the refinement of rodent cancer models.Nature protocols · 2024Review
- Adolescent intermittent ethanol exposure alters adult exploratory and affective behaviors, and cerebellar Grin2b expression in C57BL/6J mice.Drug and alcohol dependence · 2023Article
- Myelinating Glia: Potential Therapeutic Targets in Polyglutamine Spinocerebellar Ataxias.Cells · 2023Review
- The cerebellum and anxiety.Frontiers in cellular neuroscience · 2023Article
- Consensus Paper: Strengths and Weaknesses of Animal Models of Spinocerebellar Ataxias and Their Clinical Implications.Cerebellum (London, England) · 2022Article
- Calpains as novel players in the molecular pathogenesis of spinocerebellar ataxia type 17.Cellular and molecular life sciences : CMLS · 2022Article
- What is the Pathogenic CAG Expansion Length in Huntington's Disease?Journal of Huntington's disease · 2021Review
- Neuroimaging Spectrum at Pre-, Early, and Late Symptomatic Stages of SCA17 Mice.Cerebellum (London, England) · 2020Article
- Molecular determinants underlying functional innovations of TBP and their impact on transcription initiation.Nature communications · 2020Article
- Complexin I knockout rats exhibit a complex neurobehavioral phenotype including profound ataxia and marked deficits in lifespan.Pflugers Archiv : European journal of physiology · 2020Article
- Molecular Mechanisms and Therapeutics for SCA17.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2019Review
- Motor Performances of Spontaneous and Genetically Modified Mutants with Cerebellar Atrophy.Cerebellum (London, England) · 2019Review
- Inhibiting pathologically active ADAM10 rescues synaptic and cognitive decline in Huntington's disease.The Journal of clinical investigation · 2019Article
- Article
- Piperine ameliorates SCA17 neuropathology by reducing ER stress.Molecular neurodegeneration · 2018Article
- Synergistic Toxicity of Polyglutamine-Expanded TATA-Binding Protein in Glia and Neuronal Cells: Therapeutic Implications for Spinocerebellar Ataxia 17.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2017Article
- Genetically modified rodent models of SCA17.Journal of neuroscience research · 2017Review
- Molecular mechanisms underlying Spinocerebellar Ataxia 17 (SCA17) pathogenesis.Rare diseases (Austin, Tex.) · 2016Article
- Neurodevelopmental Malformations of the Cerebellar Vermis in Genetically Engineered Rats.Cerebellum (London, England) · 2015Article
- Large Polyglutamine Repeats Cause Muscle Degeneration in SCA17 Mice.Cell reports · 2015Article
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Authors and funding
11 authors at 6 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Spinocerebellar ataxia 17 (SCA17) is an autosomal-dominant, late-onset neurodegenerative disorder caused by an expanded polyglutamine (polyQ) repeat in the TATA-box-binding protein (TBP). To further investigate this devastating disease, we sought to create a first transgenic rat model for SCA17 that carries a full human cDNA fragment of the TBP gene with 64 CAA/CAG repeats (TBPQ64). In line with previous observations in mouse models for SCA17, TBPQ64 rats show a severe neurological phenotype including ataxia, impairment of postural reflexes, and hyperactivity in early stages followed by reduced activity, loss of body weight, and early death. Neuropathologically, the severe phenotype of SCA17 rats was associated with neuronal loss, particularly in the cerebellum. Degeneration of Purkinje, basket, and stellate cells, changes in the morphology of the dendrites, nuclear TBP-positive immunoreactivity, and axonal torpedos were readily found by light and electron microscopy. While some of these changes are well recapitulated in existing mouse models for SCA17, we provide evidence that some crucial characteristics of SCA17 are better mirrored in TBPQ64 rats. Thus, this SCA17 model represents a valuable tool to pursue experimentation and therapeutic approaches that may be difficult or impossible to perform with SCA17 transgenic mice. We show for the first time positron emission tomography (PET) and diffusion tensor imaging (DTI) data of a SCA animal model that replicate recent PET studies in human SCA17 patients. Our results also confirm that DTI are potentially useful correlates of neuropathological changes in TBPQ64 rats and raise hope that DTI imaging could provide a biomarker for SCA17 patients.
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