Phenotypes associated with this allele
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Sod1*G86R)M1Jwg mutation
(1 available)
|
|
|
mortality/aging
|
• die between 105 and 140 days of age
|
behavior/neurological
vision/eye
|
• light exposed eyes show shortening of the rod outer segments relative to the rod inner segments
|
|
• exposure to constant bright light for 20 days causes specific degeneration of photoreceptor cells
|
|
• light exposed eyes show a decrease in the thickness of the retinal outer nuclear layer due to loss of photoreceptor cells compared to controls
|
|
• exposure to constant bright light for 20 days causes a diminution of electroretinographic activity
|
nervous system
|
• light exposed eyes show shortening of the rod outer segments relative to the rod inner segments
|
|
• exposure to constant bright light for 20 days causes specific degeneration of photoreceptor cells
|
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Sod1*G86R)M1Jwg mutation
(1 available)
|
|
|
mortality/aging
|
• none survive past 4 months of age
(J:22628)
• compensating the energetic imbalance with a highly energetic diet extends the mean survival by 20% and improves motor neuronal function
(J:91800)
|
behavior/neurological
|
• cease food and water intake
|
|
• exhibit an age-related rapidly progressive decline of motor function
|
|
• animals show increased incidence of falling
|
|
• mice show reduced ability with aging to remain on rotating rod
|
|
• progressive decrease in grip strength is observed starting at around 15 weeks of age
|
|
• at 3-4 months of age, develop a generalized weakness that progresses within 72 hours to total immobility
|
|
• develop a spastic paralysis, initially involving the hindlimbs but progressing to the forelimb, that is associated with profound muscle wasting
|
nervous system
|
• neuron loss is also seen in the hypoglossal nucleus but it is more variable and much milder than the loss in the facial nucleus
|
|
• consistent neuronal loss in the oculomotor nucleus is seen only in the two most severely affected mice
|
|
• exhibit a variable (16-72%) decrease in neuron number in the facial nucleus and a smaller nuclear volume
|
|
• exhibit a moderate deletion of neurons in the trigeminal motor nucleus
|
|
• within the brain stem and neocortex, show degenerative changes in the motor components of cranial nerve nuclei
|
|
• exhibit a few dystrophic neurites in the dorsal horn, however do not detect any argyrophilic perikarya
|
|
• exhibit moderate to severe degenerative changes within the ventral horns
• exhibit numerous dystrophic neurites in the ventral horn gray matter, large and small argyrophilic perikarya, and swollen fragmented processes
|
|
• exhibit neurodegeneration of motorneurons within the spinal cord, brain stem, and neocortex and show some degenerative changes in the hypothalamus, deep layers of the superior colliculus, deep cerebellar nuclei, basal ganglia, and thalamus
|
|
• exhibit pronounced loss of large spinal motorneurons, with remaining motorneurons showing pyknosis and karyorrhexis
• exhibit degeneration of a few cortical motorneurons in layer V
|
muscle
|
• exhibit progressive muscle wasting
|
|
• exhibit skeletal muscle hypermetabolism as indicated by increased muscle expression of genes involved in lipid and carbohydrate metabolism
|
adipose tissue
|
• exhibit reduced adipose tissue accumulation
|
|
• seen in both asymptomatic and symptomatic homozygotes
|
|
• epididymal and retroperitoneal white adipose tissue is markedly reduced or almost nonexistent in the asymptomatic phase of the disease
|
growth/size/body
|
• body mass and body weight is lower in symptomatic (75 days of age) and early symptomatic (105 days of age) mutants
|
|
• mice show decrease in body weight compared to controls from 14-16 weeks after birth onwards
|
homeostasis/metabolism