Article
PABPN1 suppresses TDP-43 toxicity in ALS disease models.
Human molecular genetics - 15 Sept 2015
Chou Ching-Chieh, Alexeeva Olga M, Yamada Shizuka, Pribadi Amy, Zhang Yi, Mo Bi, Williams Kathryn R, Zarnescu Daniela C, Rossoll Wilfried
Abstract excerpt
TAR DNA-binding protein 43 (TDP-43) is a major disease protein in amyotrophic lateral sclerosis (ALS) and related neurodegenerative diseases. Both the cytoplasmic accumulation of toxic ubiquitinated and hyperphosphorylated TDP-43 fragments and the loss of normal TDP-43 from the nucleus may contribute to the disease progression by impairing normal RNA and protein homeostasis. Therefore, both the removal of...
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