Analysis Tools
growth/size/body
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• mice show a progressive decrease in body weight with an earlier onset and more severe course in females than males
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behavior/neurological
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• mice exhibit a motor impairment on the accelerating rotarod test as early as 3 months of age in females and 6 months of age in males
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• females, but not males, exhibit decreased limb muscle strength in the hang test
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cardiovascular system
small heart
(
J:177846
)
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• heart size is reduced in 12 month old mice
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• left ventricular ejection fraction is decreased in 12 month old mice
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cellular
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• tibialis anterior muscle mitochondria show mild swelling in 8 month old females
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• tibialis anterior muscle mitochondria exhibit cristae disorganization in 8 month old females
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• mitochondria require less of the uncoupler SF6847 to become fully uncoupled than controls indicating a higher sensitivity to uncoupling and reduced respiratory capacity of mitochondria
• the calcium accumulation and retention capacity of brain mitochondria is lower than in controls at 12 months of age
• activity of COX, the terminal enzyme of the respiratory chain, is reduced by about 50% in the spinal cord of 12 month old mice
• however, brain mitochondria from 12 month old mice do not show differences in the rates of resting (state 4) and phosphorylating (state3) respiration
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immune system
dermatitis
(
J:177846
)
|
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• severe ulcerative dermatitis with tail scabbing in females by 8 months of age
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integument
dermatitis
(
J:177846
)
|
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• severe ulcerative dermatitis with tail scabbing in females by 8 months of age
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muscle
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• left ventricular ejection fraction is decreased in 12 month old mice
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• skeletal muscles are smaller at 8 months of age
• both oxidative (type 1) and glycolytic (type II) fibers in tibialis anterior muscle are smaller
• however, no differences in the number of fibers are seen and no muscle fiber necrosis or regeneration is observed
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• skeletal muscles weight about 30-40% less than controls at 8 months of age
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• muscle atrophy, especially in the hind limbs, is seen at 8 months of age
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nervous system
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• brains are smaller in 8 month old mice
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• 8 month old brains weight about 20% less than controls
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• brains show a decrease in the density of neurons in the cortex region that include the motor cortex indicating neuronal loss
• however, no abnormalities are seen in the striatum or cerebellum
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• moderate increase in GFAP staining in the lumbar spinal cord at 8 months of age, suggesting mild reactive gliosis
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• 8 month old spinal cord shows a decrease in motor neuron cell bodies, showing an approximate 35% decrease in the counts of motor neurons with a diameter larger than 30 um in the anterior horn of lumbar spinal cords
• however, no overt loss of neuromuscular junctions or denervation is seen in the tibialis anterior and diaphragm muscles
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renal/urinary system
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• penis prolapse by 8 months of age
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reproductive system
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• penis prolapse by 8 months of age
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skeleton
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| amyotrophic lateral sclerosis type 1 | DOID:0060193 |
OMIM:105400 |
J:177846 | |
