Open AccessAmyotrophic Lateral Sclerosis Model Derived from Human Embryonic Stem Cells Overexpressing Mutant Superoxide Dismutase 1
Author(s) -
Wada Tamaki,
Goparaju Sravan K.,
Tooi Norie,
Inoue Haruhisa,
Takahashi Ryosuke,
Nakatsuji Norio,
Aiba Kazuhiro
Publication year - 2012
Publication title -
stem cells translational medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.781
H-Index - 71
eISSN - 2157-6580
pISSN - 2157-6564
DOI - 10.5966/sctm.2011-0061
Subject(s) - sod1 , amyotrophic lateral sclerosis , biology , neurodegeneration , embryonic stem cell , induced pluripotent stem cell , microbiology and biotechnology , superoxide dismutase , mutant , cancer research , genetics , disease , gene , pathology , medicine , biochemistry , oxidative stress
An in vitro familial amyotrophic lateral sclerosis (FALS) model was established from human ESCs overexpressing either a wild‐type or a mutant SOD1 (G93A) gene, and the phenotypes and survival of the spinal motor neurons were evaluated. This model is expected to help unravel the disease mechanisms involved in the development of FALS and also lead to potential drug discoveries based on the prevention of neurodegeneration.