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Phenotypes Associated with This Genotype
Genotype
MGI:4829786
Allelic
Composition
Tg(SOD1*G93A)1Gur/0
Genetic
Background
B6SJL-Tg(SOD1*G93A)1Gur
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(SOD1*G93A)1Gur mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• about 20% lower glycinergic synapses (GlyT2-boutons) densities on lumbar spinal motoneurons in asymptomatic 8-week-old mice (J:144199)
• the density of GlyT2-boutons shows a slight recovery at 10 weeks of age compared with 8-week-old transgenic mice (J:144199)
• the number of GlyT2-boutons is reduced further at 12 and 14 weeks of age (J:144199)
• majority of GlyT2-boutons is lost at 16 weeks of age (J:144199)
• decreased number of Renshaw cells at 12 and 14 weeks of age (J:144199)
• about 20% lower glycinergic synapses (GlyT2-boutons) densities on lumbar spinal motoneurons in asymptomatic 8-week-old mice (J:144199)
• the density of GlyT2-boutons shows a slight recovery at 10 weeks of age compared with 8-week-old transgenic mice (J:144199)
• the number of GlyT2-boutons is reduced further at 12 and 14 weeks of age (J:144199)
• majority of GlyT2-boutons is lost at 16 weeks of age (J:144199)
• decreased number of Renshaw cells at 12 and 14 weeks of age (J:144199)
• decreased density of ChAT-boutons at 16 weeks of age (J:144199)
• decreased density of ChAT-boutons at 16 weeks of age (J:144199)
• reduction in dendrite length and branch nodes on basal dendrites of prelimbic/infralimbic medial prefrontal cortex neurons (J:149044)
• smaller diameter of basal dendrites on branch order one (J:149044)
• reduction in dendrite length and branch nodes on basal dendrites of prelimbic/infralimbic medial prefrontal cortex neurons (J:149044)
• smaller diameter of basal dendrites on branch order one (J:149044)
• decreased spine density on basal dendrite segments of branch order four (J:149044)
• decreased spine density on basal dendrite segments of branch order four (J:149044)
• cytoplasmic vacuoles in some motoneuron cell bodies and proximal dendrites in lumbar spinal cord at 10 weeks of age (J:144199)
• more apparent cytoplasmic vacuoles in motoneurons and more motoneurons possessed vacuoles at 12 weeks of age (J:144199)
• cytoplasmic vacuoles in some motoneuron cell bodies and proximal dendrites in lumbar spinal cord at 10 weeks of age (J:144199)
• more apparent cytoplasmic vacuoles in motoneurons and more motoneurons possessed vacuoles at 12 weeks of age (J:144199)
• cytoplasmic vacuoles starting at 10 weeks of age (J:144199)
• swollen, degenerating axons with segmental enlargements in the ventral horn neuropil and white matter starting at 10 weeks of age (J:144199)
• the residual motoneurons tend to be smaller than that the typical alpha-motoneurons at 16 weeks of age (J:144199)
• the average size of motoneurons gradually increases from 10 weeks of age and reaches maximum at 12 weeks (J:144199)
• decreased number of mid-size (20-30 micrometer) motoneurons at 14 weeks of age (J:144199)
• cytoplasmic vacuoles starting at 10 weeks of age (J:144199)
• swollen, degenerating axons with segmental enlargements in the ventral horn neuropil and white matter starting at 10 weeks of age (J:144199)
• the residual motoneurons tend to be smaller than that the typical alpha-motoneurons at 16 weeks of age (J:144199)
• the average size of motoneurons gradually increases from 10 weeks of age and reaches maximum at 12 weeks (J:144199)
• decreased number of mid-size (20-30 micrometer) motoneurons at 14 weeks of age (J:144199)
• decreased number of motoneurons at 14 weeks of age (J:144199)
• the number of motoneurons is reduced further at 16 weeks of age (J:144199)
• decreased number of motoneurons at 14 weeks of age (J:144199)
• the number of motoneurons is reduced further at 16 weeks of age (J:144199)
• swollen, degenerating axons with segmental enlargements in the ventral horn neuropil at 10 weeks of age (J:144199)
• decreased number of motoneurons at 14 weeks of age (J:144199)
• the number of motoneurons is reduced further at 16 weeks of age (J:144199)
• swollen, degenerating axons with segmental enlargements in the ventral horn neuropil at 10 weeks of age (J:144199)
• decreased number of motoneurons at 14 weeks of age (J:144199)
• the number of motoneurons is reduced further at 16 weeks of age (J:144199)
• about 20% lower GlyT2-boutons densities on lumbar spinal motoneurons in asymptomatic 8-week-old mice (J:144199)
• the density of GlyT2-boutons shows a slight recovery at 10 weeks of age compared with 8-week-old transgenic mice (J:144199)
• the number of GlyT2-boutons is reduced further at 12 and 14 weeks of age (J:144199)
• majority of GlyT2-boutons is lost at 16 weeks of age (J:144199)
• decreased number of Renshaw cells at 12 and 14 weeks of age (J:144199)
• decreased density of ChAT-boutons at 16 weeks of age (J:144199)
• about 20% lower GlyT2-boutons densities on lumbar spinal motoneurons in asymptomatic 8-week-old mice (J:144199)
• the density of GlyT2-boutons shows a slight recovery at 10 weeks of age compared with 8-week-old transgenic mice (J:144199)
• the number of GlyT2-boutons is reduced further at 12 and 14 weeks of age (J:144199)
• majority of GlyT2-boutons is lost at 16 weeks of age (J:144199)
• decreased number of Renshaw cells at 12 and 14 weeks of age (J:144199)
• decreased density of ChAT-boutons at 16 weeks of age (J:144199)
• swollen, degenerating axons with segmental enlargements in the ventral horn neuropil at 10 weeks of age (J:144199)
• swollen, degenerating axons with segmental enlargements in the ventral horn neuropil at 10 weeks of age (J:144199)
• swollen, degenerating axons with segmental enlargements in the white matter at 10 weeks of age (J:144199)
• swollen, degenerating axons with segmental enlargements in the white matter at 10 weeks of age (J:144199)

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 (J:144199)
• fulminant mitochondrial swelling at 12 weeks of age (J:144199)
• 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 (J:144199)
• fulminant mitochondrial swelling at 12 weeks of age (J:144199)

behavior/neurological
• impaired extinction of conditioned fear (J:149044)
• a stronger amount of freezing and run a shorter distance in comparison with wild-type following the three tone alone presentations on day 3 (J:149044)
• impaired extinction of conditioned fear (J:149044)
• a stronger amount of freezing and run a shorter distance in comparison with wild-type following the three tone alone presentations on day 3 (J:149044)

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


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
02/02/2016
MGI 6.02
The Jackson Laboratory