Modeling ALS with iPSCs Reveals that Mutant SOD1 Misregulates Neurofilament Balance in Motor Neurons

Chen, Hong; Qian, Kun; Du, Zhongwei; Cao, Jingyuan; Petersen, Andrew; Liu, Huisheng; Blackbourn, Lisle W.; Huang, CindyTzu-Ling; Errigo, Anthony; Yin, Yingnan; Lu, Jianfeng; Ayala, Melvin; Zhang, Su-Chun

doi:10.1016/j.stem.2014.02.004

Modeling ALS with iPSCs Reveals that Mutant SOD1 Misregulates Neurofilament Balance in Motor Neurons

SUMMARY 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 th…