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Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND) or Lou Gehrig’s disease, is an adult-onset, progressive, neurological disease that results in the destruction of neurons controling voluntary muscles, namely those in the motor cortex, brainstem, and spinal cord.
The pathological hallmark of ALS is the presence of inclusion bodies (abnormal aggregations of protein) in the cytoplasm of motor neurons. In the vast majority of cases, the main component of the inclusion bodies is TDP-43 protein, however, in those with SOD1 or FUS mutations, the main component is SOD1 protein or FUS protein, respectively1,2.
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1. Saberi, S., Stauffer, J.E., Schulte, D.J., Ravits, J. Neuropathology of amyotrophic lateral sclerosis and its variants. Neurol Clin. 33, 855–876 (2015).
2. Blokhuis, A.M., Groen, E.J., Koppers, M., van den Berg, L.H., Pasterkamp, R.J. Protein aggregation in amyotrophic lateral sclerosis. Acta Neuropathol. 125, 777–794 (2013).
3. Evans, C.S., Holzbaur, E.L.F. Autophagy and mitophagy in ALS. Neurobiol. Dis. 122, 35–40 (2019).