title: Motor neurons derived from ALS-related mouse iPS cells recapitulate pathological features of ALS.
abstract: Amyotrophic lateral sclerosis (ALS) is a late-onset progressive neurodegenerative disease characterized by the loss of motor neurons in the spinal cord and brain. Mutations in Cu/Zn superoxide dismutase 1 (SOD1) are known to induce ALS. Although many research models have been developed, the exact pathological mechanism of ALS remains unknown. The recently developed induced pluripotent stem (iPS) cell technology is expected to illuminate the pathological mechanisms and new means of treatment for neurodegenerative diseases. To determine the pathological mechanism of ALS, we generated mouse iPS (miPS) cells from experimental ALS transgenic mice and control mice and characterized the cells using molecular biological methods. The generated miPS cells expressed many pluripotent genes and differentiated into three germ layers in vitro and in vivo. Motor neurons derived from ALS-related miPS cells recapitulated the pathological features of ALS. The ALS-model motor neurons showed SOD1 aggregates, as well as decreased cell survival rate and neurite length compared with wild-type motor neurons. Our study will be helpful in revealing the mechanism of motor neuronal cell death in ALS.
authors.affiliation: Division of Life Sciences, College of Life Sciences and Biotechnology, Korea University, Seoul, Republic of Korea.
authors.forename: Ju-Hwang
authors.initials: JH
authors.lastname: Park
authors.affiliation: Department of Integrated Biomedical and Life Science, College of Health Science, Korea University, Seoul, Republic of Korea.
authors.forename: Hang-Soo
authors.initials: HS
authors.lastname: Park
authors.affiliation: Department of Integrated Biomedical and Life Science, College of Health Science, Korea University, Seoul, Republic of Korea.
authors.forename: Sunghoi
authors.initials: S
authors.lastname: Hong
authors.affiliation: Division of Life Sciences, College of Life Sciences and Biotechnology, Korea University, Seoul, Republic of Korea.
authors.forename: Seongman
authors.initials: S
authors.lastname: Kang
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology; D000818:Animals; D002478:Cells, Cultured; D006801:Humans; D057026:Induced Pluripotent Stem Cells / Q000473:pathology*; D051379:Mice; D008822:Mice, Transgenic; D009046:Motor Neurons / Q000473:pathology*; D009154:Mutation; D055495:Neurogenesis; D000072105:Superoxide Dismutase-1 / Q000235:genetics
chemical_list: C000606290:SOD1 protein, human; D000072105:Superoxide Dismutase-1