Article
FUS transgenic rats develop the phenotypes of amyotrophic lateral sclerosis and frontotemporal lobar degeneration.
PLoS genetics - 1 Mar 2011
Huang Cao, Zhou Hongxia, Tong Jianbin, Chen Han, Liu Yong-Jian, Wang Dian, Wei Xiaotao, Xia Xu-Gang
Abstract excerpt
Fused in Sarcoma (FUS) proteinopathy is a feature of frontotemporal lobar dementia (FTLD), and mutation of the fus gene segregates with FTLD and amyotrophic lateral sclerosis (ALS). To study the consequences of mutation in the fus gene, we created transgenic rats expressing the human fus gene with or without mutation. Overexpression of a mutant (R521C substitution), but not normal, human FUS induced progressive...
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