Elucidating the mechanism of aggregate formation of ALS/FTD-causative TDP-43 and its toxicity

A research group led by Dr. Akira Kitamura, lecturer at Faculty of Advanced Life Science, Hokkaido University, Ms. Ai Fujimoto, doctoral student at Graduate School of Life Science, Hokkaido University, and Dr. Richard Morimoto, Professor in Northwestern University, USA, elucidated the intracellular aggregate formation mechanism of TDP-43 carboxy-terminal fragment, which causes amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), and demonstrated toxicity of the aggregate using nematode worms (C. elegans), a model organism.

Original article: Hetero-oligomerization of TDP-43 carboxy-terminal fragments with cellular proteins contributes to proteotoxicity