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Significance TDP-43 is the defining pathological hallmark protein for two overlapping adult-onset neurodegenerative diseases, amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). TDP-43 proteinopathies are also found in other major neurodegenerative diseases, such as Alzheimer’s disease, further highlighting the pivotal role of TDP-43 in the nervous system. Curiously, TDP-43 aggregates are also found in oligodendrocytes, which provide myelination and metabolic support for the neurons. Here, we show that TDP-43 is indispensable, in a cell-autonomous manner, for the proper functioning of mature oligodendrocytes, in particular, myelination and cell survival. Specifically, TDP-43 depletion leads to RIPK1-mediated necroptosis of mature oligodendrocytes and down-regulation of myelin proteins that are essential for myelination but exerts no apparent toxicity on motor neurons.

Cell-autonomous requirement of TDP-43, an ALS/FTD signature protein, for oligodendrocyte survival and myelination