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Phenotypes Associated with This Genotype
Genotype
MGI:5749248
Allelic
Composition
Sod1m1H/Sod1m1H
Genetic
Background
involves: C3H/HeH * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Sod1m1H mutation (1 available); any Sod1 mutation (15 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
adipose tissue
• mice show less fat mass at 52 weeks of age

behavior/neurological
• females exhibit a sensory deficit in response to a low threshold mechanical stimulus
• however, females do not show deficits toward a high-threshold mechanical stimulus
• mice develop tremors with age
• mice show impaired performance on the accelerating rotarod, with females showing earlier deficits (23 weeks) than males (67 weeks)
• mice exhibit reduced grip strength from 6 weeks of age
• ability to walk down a vertical wire grate (negative geotaxis) is impaired
• mice develop gait abnormalities with age
• females exhibit a sensory deficit in response noxious heat stimulus
• however, females do not show deficits toward a noxious cold stimulus (cold plate)

growth/size/body
• mice show less fat mass at 52 weeks of age
• reduction in body weight from 4 weeks of age

hematopoietic system
• microgliosis is seen in the lumbar spinal cord at 15 weeks of age and increases further at 52 weeks of age

immune system
• microgliosis is seen in the lumbar spinal cord at 15 weeks of age and increases further at 52 weeks of age
• liver at autopsy shows moderate parenchymal inflammation

integument
• females exhibit a sensory deficit in response to a low threshold mechanical stimulus
• however, females do not show deficits toward a high-threshold mechanical stimulus
• females exhibit a sensory deficit in response noxious heat stimulus
• however, females do not show deficits toward a noxious cold stimulus (cold plate)

liver/biliary system
• liver at autopsy shows moderate parenchymal inflammation
• liver at autopsy shows diffuse nuclear anisocytosis, moderate parenchymal inflammation and patchy fatty change
• mice exhibit more liver tumors at autopsy than wild-type mice

mortality/aging
• fewer than the expected numbers of homozygotes are obtained
• shorter survival, with males having a significantly reduced lifespan compared to females (495 days vs. 588 days, respectively)

muscle
• mice exhibit changes in the histochemical phenotype of fast twitch muscles indicative of an oxidative phenotype that is more characteristic of slow twitch, type I muscle fibers
• progressive loss in muscle strength between 15 and 52 weeks of age, with weaker tibialis anterior muscle at 15 weeks that declines further at 52 weeks, and a reduction in muscle force of EDL by about 35% in 52 week old females
• the fatigue index of EDL is normal at 15 weeks of age but is increased by 30% at 52 weeks of age

nervous system
• microgliosis is seen in the lumbar spinal cord at 15 weeks of age and increases further at 52 weeks of age
• astrogliosis is seen in the lumbar spinal cord at 15 weeks of age and increases further at 52 weeks of age
• mice exhibit an increase in denervated endplate neuromuscular junctions in the extensor digitorum longus (EDL) at 52, but not 15, weeks of age indicating distal progressive denervation of the EDL muscle
• mice show signs of cellular degeneration of corticospinal motor neurons in layer V of the motor cortex at 29 weeks of age
• corticospinal motor neuron numbers are normal at 15 weeks of age but are reduced by 22% at 29 weeks of age
• mice develop lower motor neuron 9in the lumbar spinal cord) degeneration between 6 and 15 weeks of age, showing a 23% reduction in the number of lower motor neurons at 15 weeks of age, which remains stable at 52 weeks of age
• mice develop upper motor neuron degeneration between 15 and 29 weeks of age, with a 22% reduction in corticospinal motor neuron numbers at 29 weeks

skeleton
• mice become severely kyphotic with age

neoplasm
• mice exhibit more liver tumors at autopsy than wild-type mice

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
amyotrophic lateral sclerosis type 1 DOID:0060193 OMIM:105400
J:219360


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
02/16/2021
MGI 6.16
The Jackson Laboratory