Article
The influence of pathological mutations and proline substitutions in TDP-43 glycine-rich peptides on its amyloid properties and cellular toxicity.
PloS one - 1 Jan 2014
Sun Chia-Sui, Wang Cindy Yu-Hsiang, Chen Bryan Po-Wen, He Ruei-Yu, Liu Gerard Chun-Hao, Wang Chih-Hsien, Chen Wenlung, Chern Yijuang, Huang Joseph Jen-Tse
Abstract excerpt
TAR DNA-binding protein (TDP-43) was identified as the major ubiquitinated component deposited in the inclusion bodies in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U) in 2006. Later on, numerous ALS-related mutations were found in either the glycine or glutamine/asparagine-rich region on the TDP-43 C-terminus, which hinted on the importance...
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