Article
Divergent phenotypes in mutant TDP-43 transgenic mice highlight potential confounds in TDP-43 transgenic modeling.
PloS one - 1 Jan 2014
D'Alton Simon, Altshuler Marcelle, Cannon Ashley, Dickson Dennis W, Petrucelli Leonard, Lewis Jada
Abstract excerpt
The majority of cases of frontotemporal lobar degeneration and amyotrophic lateral sclerosis are pathologically defined by the cleavage, cytoplasmic redistribution and aggregation of TAR DNA binding protein of 43 kDa (TDP-43). To examine the contribution of these potentially toxic mechanisms in vivo, we generated transgenic mice expressing human TDP-43 containing the familial amyotrophic lateral sclerosis-linked...
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