Models of multiple system atrophy

Current Topics in Behavioral Neurosciences
Lisa FellnerNadia Stefanova

Abstract

Multiple system atrophy (MSA) is a predominantly sporadic, adult-onset, fatal neurodegenerative disease of unknown etiology. MSA is characterized by autonomic failure, levodopa-unresponsive parkinsonism, cerebellar ataxia and pyramidal signs in any combination. MSA belongs to a group of neurodegenerative disorders termed α-synucleinopathies, which also include Parkinson's disease and dementia with Lewy bodies. Their common pathological feature is the occurrence of abnormal α-synuclein positive inclusions in neurons or glial cells. In MSA, the main cell type presenting aggregates composed of α-synuclein are oligodendroglial cells . This pathological hallmark, also called glial cytoplasmic inclusions (GCIs) , is associated with progressive and profound neuronal loss in various regions of the brain. The development of animal models of MSA is justified by the limited understanding of the mechanisms of neurodegeneration and GCIs formation, which is paralleled by a lack of therapeutic strategies. Two main types of rodent models have been generated to replicate different features of MSA neuropathology. On one hand, neurotoxin-based models have been produced to reproduce neuronal loss in substantia nigra pars compacta and striatum. On ...Continue Reading

Citations

Mar 15, 2015·Neurobiology of Disease·Dominik BrückLisa Fellner
Apr 22, 2016·Journal of Neural Transmission·Kurt A Jellinger, Gregor K Wenning
Jun 7, 2017·Neurotherapeutics : the Journal of the American Society for Experimental NeuroTherapeutics·Franziska RichterGal Bitan
Oct 16, 2015·The Cerebellum·David J LinJeremy D Schmahmann

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