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• W1973365424 abstract "Multiple system atrophy (MSA) is a rare neurodegenerative disease of undetermined cause manifesting with progressive autonomic failure (AF), cerebellar ataxia and parkinsonism due to neuronal loss in multiple brain areas associated with (oligodendro)glial cytoplasmic α-synuclein (αSYN) inclusions (GCIs). Using proteolipid protein (PLP)-α-synuclein (αSYN) transgenic mice we have previously reported parkinsonian motor deficits triggered by MSA-like αSYN inclusions. We now extend these observations by demonstrating degeneration of brain areas that are closely linked to progressive AF and other non-motor symptoms in MSA, in (PLP)-αSYN transgenic mice as compared to age-matched non-transgenic controls. We show delayed loss of cholinergic neurons in nucleus ambiguus at 12 months of age as well as early neuronal loss in laterodorsal tegmental nucleus, pedunculopontine tegmental nucleus and Onuf's nucleus at 2 months of age associated with αSYN oligodendroglial overexpression. We also report that neuronal loss triggered by MSA-like αSYN inclusions is absent up to 12 months of age in the thoracic intermediolateral cell column suggesting a differential dynamic modulation of αSYN toxicity within the murine autonomic nervous system. Although the spatial and temporal evolution of central autonomic pathology in MSA is unknown our findings corroborate the utility of the (PLP)-αSYN transgenic mouse model as a testbed for the study of oligodendroglial αSYN mediated neurodegeneration replicating both motor and non-motor aspects of MSA." @default.
• W1973365424 created "2016-06-24" @default.
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• W1973365424 date "2010-08-01" @default.
• W1973365424 modified "2023-10-14" @default.
• W1973365424 title "Targeted overexpression of human α-synuclein in oligodendroglia induces lesions linked to MSA -like progressive autonomic failure" @default.
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• W1973365424 doi "https://doi.org/10.1016/j.expneurol.2010.05.008" @default.
• W1973365424 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/2913120" @default.
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