Induced pluripotent stem cells as models for Amyotrophic Lateral Sclerosis

Abstract About 10% of Amyotrophic Lateral Sclerosis (ALS) cases can be explained by mutation in a gene. Human induced pluripotent stem cells enable to conduct research of ALS using authentic human relevant cells. This chapter will cover the following issues: Cells that are relevant for modeling ALS (motor neurons, microglia, astrocytes, cortical cells, Schwann cells); biochemical and physiological assays relevant to ALS symptoms (oxidative stress, electric activity, protein aggregates, inflammation, spine and dendritic structure); autophagy; senescence; potential targets for drug discovery. Knowledge on a mutated gene or on a group of sporadic ALS cases that have a common phenotype is transferred immediately into research models. Sophisticated methods detecting the distribution of mutated proteins are incorporated very quickly into research. A new hypothesis, like neuro-inflammation by nonneuronal neighboring cells, is immediately implemented in ALS research models. The urgent need to find a cure for ALS contributes to the dynamic of ALS research field.