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Neuronal Alpha-Synucleinopathy With Severe Movement Disorder in Mice Expressing A53T Human Alpha-Synuclein

Neuronal Alpha-Synucleinopathy With Severe Movement Disorder in Mice Expressing A53T Human Alpha-Synuclein

Benoit I Giasson, John E Duda, Shawn M Quinn, Bin Zhang, John Q Trojanowski, Virginia M-Y Lee

Neuron. 2002 May 16;34(4):521-33.

PMID: 12062037

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.

Chemicals Related in the Paper:

Catalog Number	Product Name	Structure	CAS Number	Price
IAR4248204	α-Synuclein A53T human			Price

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