Mouse Genome Informatics
tg
    Tg(SOD1*G93A)1Gur/0
B6SJL-Tg(SOD1*G93A)1Gur
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
nervous system
• about 20% lower glycinergic synapses (GlyT2-boutons) densities on lumbar spinal motoneurons in asymptomatic 8-week-old mice
• the density of GlyT2-boutons shows a slight recovery at 10 weeks of age compared with 8-week-old transgenic mice
• the number of GlyT2-boutons is reduced further at 12 and 14 weeks of age
• majority of GlyT2-boutons is lost at 16 weeks of age
• decreased number of Renshaw cells at 12 and 14 weeks of age
• decreased density of ChAT-boutons at 16 weeks of age
• reduction in dendrite length and branch nodes on basal dendrites of prelimbic/infralimbic medial prefrontal cortex neurons
• smaller diameter of basal dendrites on branch order one
• decreased spine density on basal dendrite segments of branch order four
• cytoplasmic vacuoles in some motoneuron cell bodies and proximal dendrites in lumbar spinal cord at 10 weeks of age
• more apparent cytoplasmic vacuoles in motoneurons and more motoneurons possessed vacuoles at 12 weeks of age
• cytoplasmic vacuoles starting at 10 weeks of age
• swollen, degenerating axons with segmental enlargements in the ventral horn neuropil and white matter starting at 10 weeks of age
• the residual motoneurons tend to be smaller than that the typical alpha-motoneurons at 16 weeks of age
• the average size of motoneurons gradually increases from 10 weeks of age and reaches maximum at 12 weeks
• decreased number of mid-size (20-30 micrometer) motoneurons at 14 weeks of age
• decreased number of motoneurons at 14 weeks of age
• the number of motoneurons is reduced further at 16 weeks of age
• swollen, degenerating axons with segmental enlargements in the ventral horn neuropil at 10 weeks of age
• decreased number of motoneurons at 14 weeks of age
• the number of motoneurons is reduced further at 16 weeks of age
• about 20% lower GlyT2-boutons densities on lumbar spinal motoneurons in asymptomatic 8-week-old mice
• the density of GlyT2-boutons shows a slight recovery at 10 weeks of age compared with 8-week-old transgenic mice
• the number of GlyT2-boutons is reduced further at 12 and 14 weeks of age
• majority of GlyT2-boutons is lost at 16 weeks of age
• decreased number of Renshaw cells at 12 and 14 weeks of age
• decreased density of ChAT-boutons at 16 weeks of age
• swollen, degenerating axons with segmental enlargements in the ventral horn neuropil at 10 weeks of age
• swollen, degenerating axons with segmental enlargements in the white matter at 10 weeks of age

cellular
• vacuoles in large swollen mitochondria in the cell bodies and proximal dendrites of some motoneurons and in dendrites within in the neuropil at 10 weeks of age
• fulminant mitochondrial swelling at 12 weeks of age

behavior/neurological
• impaired extinction of conditioned fear
• a stronger amount of freezing and run a shorter distance in comparison with wild-type following the three tone alone presentations on day 3

Mouse Models of Human Disease
OMIM IDRef(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:144199