Ontology highlight
ABSTRACT:
SUBMITTER: Yamashita T
PROVIDER: S-EPMC5206745 | biostudies-literature | 2017 Jan
REPOSITORIES: biostudies-literature
Yamashita Takenari T Aizawa Hitoshi H Teramoto Sayaka S Akamatsu Megumi M Kwak Shin S
Scientific reports 20170103
Nuclear dysfunction in motor neurons has been hypothesized to be a principal cause of amyotrophic lateral sclerosis (ALS) pathogenesis. Here, we investigated the mechanism by which the nuclear pore complex (NPC) is disrupted in dying motor neurons in a mechanistic ALS mouse model (adenosine deaminase acting on RNA 2 (ADAR2) conditional knockout (AR2) mice) and in ALS patients. We showed that nucleoporins (Nups) that constituted the NPC were cleaved by activated calpain via a Ca<sup>2+</sup>-perm ...[more]