title: Boundary Cap Neural Crest Stem Cells Promote Survival of Mutant SOD1 Motor Neurons.
abstract: ALS is a devastating disease resulting in degeneration of motor neurons (MNs) in the brain and spinal cord. The survival of MNs strongly depends on surrounding glial cells and neurotrophic support from muscles. We previously demonstrated that boundary cap neural crest stem cells (bNCSCs) can give rise to neurons and glial cells in vitro and in vivo and have multiple beneficial effects on co-cultured and co-implanted cells, including neural cells. In this paper, we investigate if bNCSCs may improve survival of MNs harboring a mutant form of human SOD1 (SOD1G93A) in vitro under normal conditions and oxidative stress and in vivo after implantation to the spinal cord. We found that survival of SOD1G93AMNs in vitro was increased in the presence of bNCSCs under normal conditions as well as under oxidative stress. In addition, when SOD1G93AMN precursors were implanted to the spinal cord of adult mice, their survival was increased when they were co-implanted with bNCSCs. These findings show that bNCSCs support survival of SOD1G93AMNs in normal conditions and under oxidative stress in vitro and improve their survival in vivo, suggesting that bNCSCs have a potential for the development of novel stem cell-based therapeutic approaches in ALS models.
authors.affiliation: Department of Neuroscience, Uppsala University Biomedical Center, Box 593, 75124, Uppsala, Sweden.
authors.forename: Tanya
authors.initials: T
authors.lastname: Aggarwal
authors.affiliation: Department of Neuroscience, Uppsala University Biomedical Center, Box 593, 75124, Uppsala, Sweden.
authors.forename: Jan
authors.initials: J
authors.lastname: Hoeber
authors.affiliation: Department of Neuroscience, Uppsala University Biomedical Center, Box 593, 75124, Uppsala, Sweden.
authors.forename: Patrik
authors.initials: P
authors.lastname: Ivert
authors.affiliation: Department of Neuroscience, Uppsala University Biomedical Center, Box 593, 75124, Uppsala, Sweden.
authors.forename: Svitlana
authors.initials: S
authors.lastname: Vasylovska
authors.affiliation: Department of Neuroscience, Uppsala University Biomedical Center, Box 593, 75124, Uppsala, Sweden. elena.kozlova@neuro.uu.se.
authors.forename: Elena N
authors.initials: EN
authors.lastname: Kozlova
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000473:pathology*; D000818:Animals; D002470:Cell Survival; D002478:Cells, Cultured; D006801:Humans; D051379:Mice; D008819:Mice, Nude; D009046:Motor Neurons / Q000473:pathology*; D009154:Mutation; D009432:Neural Crest / Q000637:transplantation; D058953:Neural Stem Cells / Q000166:cytology / Q000637:transplantation; D033581:Stem Cell Transplantation; D000072105:Superoxide Dismutase-1 / Q000235:genetics
chemical_list: C000606290:SOD1 protein, human; D000072105:Superoxide Dismutase-1
keywords: Amyotrophic lateral sclerosis; Neurodegeneration; Neuroglia; Oxidative stress; Transplantation