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Abstract
The most evident phenotype of degenerative motoneuron disease is the loss of motor function which accompanies motoneuron death. In both amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA), it is now clear that dysfunction is not restricted to motoneurons but is manifest in the spinal circuits in which motoneurons are embedded. As mounting evidence shows that motoneurons possess more elaborate and extensive connections within the spinal cord than previously realized, it is necessary to consider the role of this circuitry and its dysfunction in the disease process. In this review article, we ask if the selective vulnerability of the different motoneuron types and the relative disease resistance of distinct motoneuron groups can be understood in terms of their intraspinal connections.
Highlights
Degenerative motoneuron diseases are devastating conditions whose underlying causes are poorly understood
The primary, cell-autonomous pathologies caused by the conditions are compounded by secondary effects that result from disruptions in the spinal circuitry
We will describe the sensitivity of the different motoneuron types to the diseases, and we will consider these differences in susceptibility in light of recent discoveries showing that the intraspinal connections of motoneurons are more extensive than previously appreciated (Bhumbra and Beato, 2018; Chopek et al, 2018)
Summary
The most evident phenotype of degenerative motoneuron disease is the loss of motor function which accompanies motoneuron death. In both amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA), it is clear that dysfunction is not restricted to motoneurons but is manifest in the spinal circuits in which motoneurons are embedded. As mounting evidence shows that motoneurons possess more elaborate and extensive connections within the spinal cord than previously realized, it is necessary to consider the role of this circuitry and its dysfunction in the disease process.
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