Oligodendroglial Densities and Myelin Structure Are Altered in TDP-43 Related Amyotrophic Lateral Sclerosis

Abstract

Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disease characterized by the degeneration of motor neurons. However, the surrounding glia, including oligodendrocytes, also exhibit ALS pathology and TDP-43 related dysfunction. Given that oligodendrocytes, the myelinating cells of the central nervous system, are essential for motor neuron function, they may play an underappreciated role in ALS. Here, we have extensively characterized the oligodendrocyte lineage and myelin integrity in the TDP-43Q331K mouse model of ALS. In the lumbar spinal cord of end-stage male TDP-43Q331K mice (TDP-43), compared to wild-type littermates (WT), oligodendrocyte precursor cell (OPC) density, ol..