References
Query Results -- Details
MGI Accession ID: MGI:2179902
J Number: J:76657
Other Accession IDs:
Title: Neuronal alpha-synucleinopathy with severe movement disorder in mice expressing A53T human alpha-synuclein.
Authors: Giasson BI; Duda JE; Quinn SM; Zhang B; Trojanowski JQ; Lee VM
Journal: Neuron
Volume: 34
Issue: 4
Date: 2002 May 16
Year: 2002
Pages: 521-33
Review Status: Peer Reviewed
Abstract:
alpha-Synucleinopathies are neurodegenerative disorders that range pathologically from the demise of select groups of nuclei to pervasive degeneration throughout the neuraxis. Although mounting evidence suggests that alpha-synuclein lesions lead to neurodegeneration, this remains controversial. To explore this issue, we generated transgenic mice expressing wild-type and A53T human alpha-synuclein in CNS neurons. Mice expressing mutant, but not wild-type, alpha-synuclein developed a severe and complex motor impairment leading to paralysis and death. These animals developed age-dependent intracytoplasmic neuronal alpha-synuclein inclusions paralleling disease onset, and the alpha-synuclein inclusions recapitulated features of human counterparts. Moreover, immunoelectron microscopy revealed that the alpha-synuclein inclusions contained 10-16 nm wide fibrils similar to human pathological inclusions. These mice demonstrate that A53T alpha-synuclein leads to the formation of toxic filamentous alpha-synuclein neuronal inclusions that cause neurodegeneration.
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