In other pools in the same individual, there will be minimal evidence of motor unit pathology, even at late stages of disease.
Compared with control ALS, treated ALS mice exhibited improved neuromuscular function and motor unit pathology and significantly increased life span, in particular with the systemic administration of NSCs (15%).
It is, however, a sobering example of a common intensive care unit pathology that has been neglected as a topic of informative research, and in this respect, it is not alone.
After injection of these cells into SOD1-mutated mice, NSCs engrafted and migrated into the CNS, resulting in improved neuromuscular function and motor unit pathology and a significantly prolonged life span [ 87].
Further work is clearly required to determine the time course and specific location of motor unit pathology in SMA and investigate whether synaptic defects in SMA are a cause or consequence of cell death pathway activation at the cell body.
For this analysis, we reasoned that any changes that occurred only in SMAv motor neurons compared to either WTv motor neurons, or to SMAr motor neurons, could be specifically implicated in NMJ and motor unit pathology.
Stem cell therapies may modify disease pathophysiology [ 11], slow down or halt the progression of disease, and even improve neuromuscular function and motor unit pathology, possibly by providing protective factors to surrounding cells, modulating the host immune environment, inhibiting inflammation, or even replacing injured cells [ 12– 17].
These motor-unit pathologies in mouse models imply that SMN is especially important during development, which in turn suggests that the pathogenesis of SMA has important developmental components.
Importantly, our results suggest that the late-onset paralysis reflects a tolerance of mature motor units against SMN deficiency (discussed below), although it is accompanied by motor-unit pathologies.
Sources of these data included the screening units, the pathology laboratories and surgeons.
