Open AccessInduced pluripotent stem cell-derived motor neurons from amyotrophic lateral sclerosis (ALS) patients carrying different superoxide dismutase 1 mutations recapitulate pathological features of ALS
Author(s) -
Wenchao Li,
Na Liu,
Yan Wang,
Chen Huang,
Yanfang Li,
Hao Wang,
Xiaogang Li,
Min Deng
Publication year - 2021
Publication title -
chinese medical journal/chinese medical journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.537
H-Index - 63
eISSN - 2542-5641
pISSN - 0366-6999
DOI - 10.1097/cm9.0000000000001693
Subject(s) - sod1 , induced pluripotent stem cell , amyotrophic lateral sclerosis , biology , microbiology and biotechnology , mutant , intracellular , superoxide dismutase , pathogenesis , cancer research , genetics , pathology , medicine , disease , immunology , gene , biochemistry , embryonic stem cell , oxidative stress
Investigations of the pathogenic mechanisms in motor neurons (MNs) derived from amyotrophic lateral sclerosis (ALS) disease-specific induced pluripotent stem (iPS) cell lines could improve understanding of the issues affecting MNs. Therefore, in this study we explored mutant superoxide dismutase 1 (SOD1) protein expression in MNs derived from the iPS cell lines of ALS patients carrying different SOD1 mutations.