Back to article: Role of RNA Binding Proteins with prion-like domains in muscle and neuromuscular diseases
FIGURE 2: TDP43 and FUS alterations in neuromuscular diseases. Mutant TDP43 aggregates are found in motor neurons and muscles of patients. TDP-43 mislocalization causes axonopathy and mitochondrial alterations, alters synaptic transmission, NMJ development and muscle regeneration. Similar observations are made for FUS, which is also involved in subsynaptic transcription.