Ontology highlight
ABSTRACT:
SUBMITTER: Chen H
PROVIDER: S-EPMC4230530 | biostudies-literature | 2014 Jun
REPOSITORIES: biostudies-literature
Chen Hong H Qian Kun K Du Zhongwei Z Cao Jingyuan J Petersen Andrew A Liu Huisheng H Blackbourn Lisle W LW Huang CindyTzu-Ling CL Errigo Anthony A Yin Yingnan Y Lu Jianfeng J Ayala Melvin M Zhang Su-Chun SC
Cell stem cell 20140403 6
Amyotrophic lateral sclerosis (ALS) presents motoneuron (MN)-selective protein inclusions and axonal degeneration but the underlying mechanisms of such are unknown. Using induced pluripotent cells (iPSCs) from patients with mutation in the Cu/Zn superoxide dismutase (SOD1) gene, we show that spinal MNs, but rarely non-MNs, exhibited neurofilament (NF) aggregation followed by neurite degeneration when glia were not present. These changes were associated with decreased stability of NF-L mRNA and b ...[more]