Article
The pathological phenotypes of human TDP-43 transgenic mouse models are independent of downregulation of mouse Tdp-43.
PloS one - 1 Jan 2013
Xu Ya-Fei, Prudencio Mercedes, Hubbard Jaime M, Tong Jimei, Whitelaw Ena C, Jansen-West Karen, Stetler Caroline, Cao Xiangkun, Song John, Zhang Yong-Jie
Abstract excerpt
Tar DNA binding protein 43 (TDP-43) is the major component of pathological deposits in frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) and in amyotrophic lateral sclerosis (ALS). It has been reported that TDP-43 transgenic mouse models expressing human TDP-43 wild-type or ALS-associated mutations recapitulate certain ALS and FTLD pathological phenotypes. Of note, expression of human TDP-43...
Topics
- Alternative Splicing
- Amyotrophic Lateral Sclerosis
- Animals
- Animals, Newborn
- DNA-Binding Proteins
- Dendritic Spines
- Disease Models, Animal
- Down-Regulation
- Frontotemporal Lobar Degeneration
- Hemizygote