Motor Deficits in an Alpha-Synuclein Mouse Model of Parkinson's Disease are not Exacerbated by Gba1 Mutation

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Title: Motor Deficits in an Alpha-Synuclein Mouse Model of Parkinson's Disease are not Exacerbated by Gba1 Mutation
Authors: Fitzpatrick, Megan E.
Date: 2017
Abstract: Parkinson’s disease is a movement disorder characterized by nigrostriatal dopamine pathway degeneration and neuronal α-synuclein accumulation. Pathogenesis is associated with mutations in α-synuclein and Gba1 encoding alleles. Animal models created to date do not recapitulate the spectrum of clinical disease features. This thesis characterizes the bi-genic Synergy mouse, hypothesized to demonstrate motor behavioural and histological abnormalities downstream of α-synuclein overexpression and mutated Gba1. Synergy and SNCA mice (overexpressed α-synuclein with wild-type Gba1) have early onset deficits in motor coordination, muscle strength and nest building. Both exhibit increased α-synuclein concentration in the brain and cerebellar inclusions positive for two markers of pathological α-synuclein processing. Overall mutant Gba1 expression within Synergy mice does not worsen the behaviour or the histopathological findings associated with overexpression of human α-synuclein in SNCA mice. Future studies will determine whether mutant Gba1 expression alters cognitive behaviour and/or lipid homeostasis in this new bi-genic model of Parkinson’s disease.
URL: http://hdl.handle.net/10393/35689
http://dx.doi.org/10.20381/ruor-646
CollectionThèses, 2011 - // Theses, 2011 -
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