[Neuronal cells derived from induced pluripotent stem cells to model motoneuron diseases].
Among motor neuron diseases, spinal muscular atrophy type 1 and amyotrophic lateral sclerosis are very aggressive diseases with no cure. With the breakthrough of human induced pluripotent stem cells, iPS, researchers have now at their disposal a powerful tool to generate human motor neurons in culture and study the pathological defects in patient cells. In this review, we will see which tools for the study of patients motoneurons were developed from iPS cells and the different cellular models that were generated. We will also see how these models were validated and current research to identify new therapeutic leads.
Delphine
D
Bohl
D000690:Amyotrophic Lateral Sclerosis / Q000473:pathology / Q000628:therapy; D002454:Cell Differentiation; D006801:Humans; D057026:Induced Pluripotent Stem Cells / Q000502:physiology*; D008954:Models, Biological; D016472:Motor Neuron Disease / Q000473:pathology* / Q000628:therapy; D009046:Motor Neurons / Q000502:physiology*; D009134:Muscular Atrophy, Spinal / Q000473:pathology / Q000628:therapy; D058953:Neural Stem Cells / Q000502:physiology; D009457:Neuroglia / Q000502:physiology