The C-terminal TDP-43 fragments have a high aggregation propensity and harm neurons by a dominant-negative mechanism.

TAR DNA binding protein 43 KD (TDP-43) is an essential gene that regulates gene transcription, mRNA splicing and stability. In amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), two fatal neurodegenerative diseases, TDP-43 is fragmented, generating multiple fragments that include...

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Bibliographic Details
Main Authors: Chunxing Yang, Weijia Tan, Catheryne Whittle, Linghua Qiu, Lucheng Cao, Schahram Akbarian, Zuoshang Xu
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2010-12-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC3013128?pdf=render