Amyotrophic lateral sclerosis (ALS) is a fatal disease that leads to progressive degeneration of motoneurons. Mutations in the , , and genes, among others, have been associated with ALS. Although motoneuron degeneration is the common outcome of ALS, different pathological mechanisms seem to be involved in this process, depending on the genotypic background of the patient. The advent of induced pluripotent stem cell (iPSC) technology enabled the development of patient-specific cell lines, from which it is possible to generate different cell types and search for phenotypic alterations. In this review, we summarize the pathophysiological markers detected in cells differentiated from iPSCs of ALS patients. In a translational perspective, iPSCs from ALS patients could be useful for drug screening, through stratifying patients according to their genetic background.
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