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Transmission of multiple system atrophy prions to transgenic mice
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Transmission of multiple system atrophy prions to transgenic mice
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Transmission of multiple system atrophy prions to transgenic mice
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Journal Article
Transmission of multiple system atrophy prions to transgenic mice
2013
Overview
Prions are proteins that adopt alternative conformations, which become self-propagating. Increasing evidence argues that prions feature in the synucleinopathies that include Parkinson's disease, Lewy body dementia, and multiple system atrophy (MSA). Although TgM83+/+ mice homozygous for a mutant A53T α-synuclein transgene begin developing CNS dysfunction spontaneously at ∼10 mo of age, uninoculated TgM83+/- mice (hemizygous for the transgene) remain healthy. To determine whether MSA brains contain α-synuclein prions, we inoculated the TgM83+/- mice with brain homogenates from two pathologically confirmed MSA cases. Inoculated TgM83+/- mice developed progressive signs of neurologic disease with an incubation period of ∼100 d, whereas the same mice inoculated with brain homogenates from spontaneously ill TgM83+/+ mice developed neurologic dysfunction in ∼210 d. Brains of MSA-inoculated mice exhibited prominent astrocytic gliosis and microglial activation as well as widespread deposits of phosphorylated α-synuclein that were proteinase K sensitive, detergent insoluble, and formic acid extractable. Our results provide compelling evidence that α-synuclein aggregates formed in the brains of MSA patients are transmissible and, as such, are prions. The MSA prion represents a unique human pathogen that is lethal upon transmission to Tg mice and as such, is reminiscent of the prion causing kuru, which was transmitted to chimpanzees nearly 5 decades ago.
Publisher
NATIONAL ACADEMY OF SCIENCES,National Acad Sciences,National Academy of Sciences
Subject
/ alpha-Synuclein - metabolism
/ Animals
/ atrophy
/ Biological and medical sciences
/ Brain
/ dementia
/ Disease Transmission, Infectious
/ Fundamental and applied biological sciences. Psychology
/ Humans
/ infectious disease incubation period
/ kuru
/ Male
/ Mice
/ Multiple System Atrophy - metabolism
/ Multiple System Atrophy - pathology
/ mutants
/ Neurons
/ patients
/ Prions
/ Proteins
/ Rodents
