FUS Transgenic Rats Develop the Phenotypes of Amyotrophic Lateral Sclerosis and Frontotemporal Lobar Degeneration

@inproceedings{Huang2011FUSTR,
  title={FUS Transgenic Rats Develop the Phenotypes of Amyotrophic Lateral Sclerosis and Frontotemporal Lobar Degeneration},
  author={Cao Huang and Hongxia Zhou and Jianbin Tong and Han Chen and Y Liu and Dian Wang and Xiaotao Wei and Xu-Gang Xia},
  booktitle={PLoS genetics},
  year={2011}
}
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 paralysis resembling ALS. Mutant FUS transgenic rats developed progressive paralysis… CONTINUE READING

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Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis

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