Words similar to motoneuron
Example sentences for: motoneuron
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These changes include astrogliosis, fragmentation of Golgi apparatus, SOD1 aggregation and vacuolar degeneration [ 7 11 12 13 14 ] . Vacuolar degeneration is most prominent at the onset of the disease and precedes motoneuron death by two to three months [ 15 ] . Although the high expresser line of SOD1 G93Ashowed vacuoles derived from both endoplasmic reticulum and mitochondria [ 13 ] , the lower expresser line of SOD1 G93A, which more closely mimics the human SOD1 levels [ 16 ] , showed vacuoles that are nearly all developed from degenerating mitochondria [ 15 17 ] . Mitochondrial vacuolation was also reported in another transgenic line SOD1 G37R [ 7 ] .
The early onset of mitochondrial degeneration suggests that mutant SOD1 damages mitochondria and this damage plays a causative role in motoneuron degeneration.
Amyotrophic lateral sclerosis (ALS) is an age-dependent neurodegenerative disease that causes progressive motoneuron degeneration, skeletal muscle atrophy, paralysis and death [ 1 2 3 ] . Although clinically indistinguishable, the majority of cases are sporadic (SALS) and approximately 10 % are due to inherited causes (familial ALS or FALS).