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Allele Symbol Allele Name Allele ID |
Tg(Prnp-PFN1*C71G)22Zxu transgene insertion 22, Zuoshang Xu MGI:5796267 |
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| Summary |
4 genotypes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• due to paralysis
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• muscle weakness beginning at an average age of 295 days
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• reduced grip strength beginning at 121-150 days
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• impaired vertical behavior beginning at 121-150 days
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• motor neurons have reduced neurofilaments, lack a well-spread filament network and exhibit a circular distribution of neurofilaments around the cell periphery
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• reduced body weight beginning at 121-150 days
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• microgliosis is increased in ventral horn of spinal cord at the end stage of disease
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• microgliosis is increased in ventral horn of spinal cord at the end stage of disease
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• due to paralysis
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• clusters of oxidative (SDH-strong) and weak oxidative (SDH-weak) fibers as compared to interspersed distribution found in control
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• 10% of mice between 100-120 days old exhibit slight weakness (foot dragging)
• 70% of mice between 121-140 days old exhibit slight weakness
• 100% of mice between 141-170 days exhibit slight weakness, progressing to weakness (leg dragging)
• beyond 170 days, all mice progress from partial paralysis to paralysis in both fore and hind limbs
• impaired rotorod performance beginning at 121-150 days
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• microgliosis is increased in ventral horn of spinal cord at the end stage of disease
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• astrogliosis is increased in ventral horn of spinal cord at the end stage of disease
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• motor neurons have reduced neurofilaments, lack a well-spread filament network and exhibit a circular distribution of neurofilaments around the cell periphery
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• increased muscle denervation
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• motor neuron number is reduced in lumbar spinal cord at the end stage of disease (170-226 days)
• motor neuron number is reduced in the ventral horn of cervical spinal cord beginning at 132-154 days of age
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• protein aggregates are visible by day 127 and gradually accumulate until death
• motor neurons exhibit widespread small particulates in the cytoplasm and neuronal processe
• 1-2% of motor neurons form large aggregates
• strong ubiquitin staining is present in motor neurons and some neuropils
• p62/SQSTM1 staining is present as small particulates in cytoplasm of motor neurons
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• increased cellulation (and GFAP staining) in medulla
• neuronal loss is not observed in cerebral cortex, hippocampus or cerebellum
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• axon loss is progressive
• increased number of degenerating axons in the ventral root in 100-120 day old mice as compared to controls
• axon degeneration is observed in the dorsal root, but progression lags behind ventral root axons
• axon degeneration is observed in white matter of spinal cord; degeneration is mild in lateral column and more severe in ventral column
• little axon degeneration is observed in the cortical spinal track in the dorsal column
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| amyotrophic lateral sclerosis type 18 | DOID:0060209 |
OMIM:614808 |
J:235427 | |
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• heterozygote x heterozygote crosses do not produce homozygotes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• muscle weakness beginning at an average age of 700 days
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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 04/16/2024 MGI 6.23 |
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