Axonal TDP-43 Condensates Drive Neuromuscular Junction Disruption through Inhibition of Local Synthesis of Nuclear Encoded Mitochondrial Proteins

The authors showed TDP-43 accumulation in intra-muscular nerves from amyotrophic lateral sclerosis (ALS) patients and in axons of human iPSC-derived motor neurons of ALS patients, as well as in motor neurons and neuromuscular junctions of a TDP-43 mislocalization mouse model.