Models of α-synuclein aggregation in Parkinson’s disease
| Autor: | Amelia Sánchez-Capelo, Mónica Antolín-Vallespín, María Dolores Muñoz, Rosa M. Giráldez-Pérez |
|---|---|
| Přispěvatelé: | Universidad de Sevilla. Departamento de Fisiología |
| Rok vydání: | 2014 |
| Předmět: |
Parkinson's disease
Dopamine Review Protein Serine-Threonine Kinases Protein aggregation Leucine-Rich Repeat Serine-Threonine Protein Kinase-2 Pathology and Forensic Medicine Pathogenesis Mice Protein Aggregates Cellular and Molecular Neuroscience chemistry.chemical_compound α-synuclein Rotenone medicine Animals Humans Alpha-synuclein Lewy body business.industry Neurodegeneration Dopaminergic Parkinson Disease medicine.disease Rats Animal models Disease Models Animal chemistry Mutation alpha-Synuclein Parkinson’s disease Glucosylceramidase Lewy Bodies Neurology (clinical) business Neuroscience Smad3 medicine.drug |
| Zdroj: | idUS. Depósito de Investigación de la Universidad de Sevilla instname Acta Neuropathologica Communications |
| ISSN: | 2051-5960 |
| DOI: | 10.1186/s40478-014-0176-9 |
| Popis: | Parkinson’s disease (PD) is not only characterized by motor disturbances but also, by cognitive, sensory, psychiatric and autonomic dysfunction. It has been proposed that some of these symptoms might be related to the widespread pathology of α-synuclein (α-syn) aggregation in different nuclei of the central and peripheral nervous system. However, the pathogenic formation of α-syn aggregates in different brain areas of PD patients is poorly understood. Most experimental models of PD are valuable to assess specific aspects of its pathogenesis, such as toxin-induced dopaminergic neurodegeneration. However, new models are required that reflect the widespread and progressive formation of α-syn aggregates in different brain areas. Such α-syn aggregation is induced in only a few animal models, for example perikaryon inclusions are found in rats administered rotenone, aggregates with a neuritic morphology develop in mice overexpressing either mutated or wild-type α-syn, and in Smad3 deficient mice, aggregates form extensively in the perikaryon and neurites of specific brain nuclei. In this review we focus on α-syn aggregation in the human disorder, its genetics and the availability of experimental models. Indeed, evidences show that dopamine (DA) metabolism may be related to α-syn and its conformational plasticity, suggesting an interesting link between the two pathological hallmarks of PD: dopaminergic neurodegeneration and Lewy body (LB) formation. |
| Databáze: | OpenAIRE |
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