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Phenotypes Associated with This Genotype
Genotype
MGI:6286689
Allelic
Composition
Tg(Prnp-Immt/SOD1*G93A)7Gmnf/Tg(Prnp-Immt/SOD1*G93A)7Gmnf
Genetic
Background
involves: C57BL/6 * CBA * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-Immt/SOD1*G93A)7Gmnf mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
growth/size/body
• mice show a progressive decrease in body weight with an earlier onset and more severe course in females than males

behavior/neurological
• 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
• females, but not males, exhibit decreased limb muscle strength in the hang test

cardiovascular system
• heart size is reduced in 12 month old mice
• left ventricular ejection fraction is decreased in 12 month old mice

cellular
• tibialis anterior muscle mitochondria show mild swelling in 8 month old females
• tibialis anterior muscle mitochondria exhibit cristae disorganization in 8 month old females
• 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

immune system
• severe ulcerative dermatitis with tail scabbing in females by 8 months of age

integument
• severe ulcerative dermatitis with tail scabbing in females by 8 months of age

muscle
• left ventricular ejection fraction is decreased in 12 month old mice
• 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
• skeletal muscles weight about 30-40% less than controls at 8 months of age
• muscle atrophy, especially in the hind limbs, is seen at 8 months of age

nervous system
• brains are smaller in 8 month old mice
• 8 month old brains weight about 20% less than controls
• 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
• moderate increase in GFAP staining in the lumbar spinal cord at 8 months of age, suggesting mild reactive gliosis
• 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

renal/urinary system
• penis prolapse by 8 months of age

reproductive system
• penis prolapse by 8 months of age

skeleton
• hunched-back posture by 8 months of age

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
amyotrophic lateral sclerosis type 1 DOID:0060193 OMIM:105400
J:177846


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB), Gene Ontology (GO)
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last database update
02/23/2021
MGI 6.16
The Jackson Laboratory