Dependent on context, it is increasingly recognized that oligodendrocytes, aside from their normal physiological function, can be injurious or regenerative in disease states. The regenerative potential of oligodendrocyte precursor cells (OPCs) is a major focus for research into demyelinating disease. Moreover, functional perturbations in oligodendrocyte differentiation and maturation from OPCs are implicated in disorders such as multiple sclerosis and amyotrophic lateral sclerosis (ALS) 1, 2. Oligodendrocyte pathology ranging from inclusions and myelin abnormalities to reactive changes in OPCs is described in ALS 3, 4, 5. Demyelination is also present in the most common genetic cause of ALS due to a hexanucleotide intronic repeat in the C9ORF72 gene that accounts for approximately 10%–50% of familial and around 6%–10% of sporadic cases across European populations 3, 6, 7, 8. However, the differentiation and maturation potential of C9ORF72 repeat expansion‐carrying OPCs has not been investigated before. Improved mechanistic understanding of the differentiation and maturation of human oligodendrocytes from OPCs is therefore of considerable interest as a point of potential therapeutic intervention. Specifically the excitable membrane properties of oligodendrocytes that are central to their
