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Phenotypes Associated with This Genotype
involves: C3H/HeJ * C57BL/6J
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phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• mice exhibit vacuoles lesions in the olfactory bulb, the piriform cortex, several thalamic nuclei, some pons neurons, the choroid plexus, and the cerebellar roof nuclei
• at late stages
• motor neuron degeneration is observed in the ventral horns of the lumbar, thoracic, and cervical spinal cord as well as the brain stem
• degeneration is associated with vacuole formation in both dendrites and axons of motor neurons

• at 5 to 6 months of age, mice exhibit axial tremors
• at 5 to 6 months of age, mice exhibit asymmetric weakness of the limbs and when suspended by their tail exhibit difficulties extending and moving hindlimbs
• at 5 to 6 months of age, mice exhibit decreased spontaneous movement compared to wild-type mice
• eventually mice develop hindlimb paralysis

• at 5 to 6 months of age, mice exhibit muscle wasting particularly along the flanks
• mice exhibit spontaneous, positive sharp waves associated with denervation atrophy

• motor neurons exhibit mitochondrial degradation

• progressive

renal/urinary system
• mice exhibit vacuolization of epithelial cells in the proximal tubules of the kidney

• at 5 to 6 months of age

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

Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
MGI 6.08
The Jackson Laboratory