Modeling motor neuron disease: the matter of time
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Updated: Oct 13, 2026

Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells
Published on: July 29, 2016
Mandana Arbab1, Susanne Baars2, Niels Geijsen1
1Hubrecht Institute and University Medical Center (UMC) Utrecht, Utrecht, The Netherlands; Faculty of Veterinary Medicine, Utrecht University, Utrecht, The Netherlands.
Abstract:
Stem cell technologies have created new opportunities to generate unlimited numbers of human neurons in the lab and study neurodegenerative disorders such as amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA). Although some disease hallmarks have been reported in patient-derived stem cell models, it is proving more difficult to recapitulate the full phenotypic extent of these disorders. The problem with these stem cell models lies in the disparity between the advanced age of onset of neurodegenerative disorders and the embryonic nature of the in vitro derived cell types. In this review we discuss experimental methods of in vitro aging of neural cell types as a means to elicit late-onset symptoms in induced pluripotent stem cell (iPSC) models of neurodegenerative disease.

