RGD Reference Report - Potential role of α-synuclein in neurodegeneration: studies in a rat animal model. - Rat Genome Database

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Potential role of α-synuclein in neurodegeneration: studies in a rat animal model.

Authors: Stoica, George  Lungu, Gina  Bjorklund, Nicole L  Taglialatela, Giulio  Zhang, Xing  Chiu, Veronica  Hill, Herbert H  Schenk, James O  Murray, Ian 
Citation: Stoica G, etal., J Neurochem. 2012 Aug;122(4):812-22. doi: 10.1111/j.1471-4159.2012.07805.x. Epub 2012 Jun 22.
RGD ID: 42722007
Pubmed: (View Article at PubMed) PMID:22639889
DOI: Full-text: DOI:10.1111/j.1471-4159.2012.07805.x

Neuronal protein α-synuclein (α-syn) is an essential player in the development of neurodegenerative diseases called synucleinopathies. A spontaneous autosomal recessive rat model for neurodegeneration was developed in our laboratory. These rats demonstrate progressive increases in α-syn in the brain mesencephalon followed by loss of dopaminergic terminals in the basal ganglia (BG) and motor impairments. The severity of pathology is directly related to the overexpression of α-syn and parallel decrease in dopamine (DA) level in the striatum (ST) of affected rats. The neurodegeneration in this model is characterized by the presence of perikarya and neurites Lewis bodies (LB) and diffuse marked accumulation of perikaryal α-syn in the substantia nigra (SN), brain stem (BS), and striatum (ST) along with neuronal loss. Light and ultrastructural analyses revealed that the process of neuronal degeneration is a 'dying back' type. The disease process is accompanied by gliosis and release of inflammatory cytokines. This neurodegeneration is a multisystemic disease and implicate α-syn as a major factor in the pathogenesis of this inherited autosomal recessive animal model. Decrease dopamine (DA) and overexpression of α-syn in the brain mesencephalon may provide a naturally occurring animal model for Parkinson's disease (PD) and other synucleinopathies that reproduces significant pathological, neurochemical, and behavioral features of the human disease.

Annotation

Disease Annotations    

Gene Ontology Annotations    

Biological Process

Objects Annotated

Genes (Rattus norvegicus)
Myo5a  (myosin VA)

Strains
BDIV-Myo5a/StcRrrc  (NA)


Additional Information