Article
Bacterial artificial chromosome transgenic mice expressing a truncated mutant parkin exhibit age-dependent hypokinetic motor deficits, dopaminergic neuron degeneration, and accumulation of proteinase K-resistant alpha-synuclein.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 18 Feb 2009
Lu Xiao-Hong, Fleming Sheila M, Meurers Bernhard, Ackerson Larry C, Mortazavi Farzad, Lo Victor, Hernandez Daniela, Sulzer David, Jackson George R, Maidment Nigel T, Chesselet Marie-Francoise, Yang X William
Abstract excerpt
Recessive mutations in parkin are the most common cause of familial early-onset Parkinson's disease (PD). Recent studies suggest that certain parkin mutants may exert dominant toxic effects to cultured cells and such dominant toxicity can lead to progressive dopaminergic (DA) neuron degeneration in Drosophila. To explore whether mutant parkin could exert similar pathogenic effects to mammalian DA neurons in vivo,...
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