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Phenotypes Associated with This Genotype
Genotype
MGI:3814057
Allelic
Composition
Tg(SOD1*G37R)29Dpr/0
Genetic
Background
involves: C3H/HeJ * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(SOD1*G37R)29Dpr mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• numerous ubiquitin-immunoreactive, Thioflavin-S positive fibrillar protein aggregates resembling hyaline inclusions are seen in the spinal cord, ventral midbrain, and the brain stem, with fewer in the cerebellum (J:119631)
• numerous ubiquitin-immunoreactive, Thioflavin-S positive fibrillar protein aggregates resembling hyaline inclusions are seen in the spinal cord, ventral midbrain, and the brain stem, with fewer in the cerebellum (J:119631)
• at late stages (J:69178)
• at late stages (J:69178)
• motor neuron degeneration is observed in the ventral horns of the lumbar, thoracic, and cervical spinal cord as well as the brain stem (J:69178)
• degeneration is associated with vacuole formation in both dendrites and axons of motor neurons (J:69178)
• motor neuron degeneration is observed in the ventral horns of the lumbar, thoracic, and cervical spinal cord as well as the brain stem (J:69178)
• degeneration is associated with vacuole formation in both dendrites and axons of motor neurons (J:69178)

behavior/neurological
• at 6 to 8 months of age, mice exhibit axial tremors (J:69178)
• at 6 to 8 months of age, mice exhibit axial tremors (J:69178)
• at 6 to 8 months of age, mice exhibit asymmetric weakness of the limbs and when suspended by their tail exhibit difficulties extending and moving hindlimbs (J:69178)
• at 6 to 8 months of age, mice exhibit asymmetric weakness of the limbs and when suspended by their tail exhibit difficulties extending and moving hindlimbs (J:69178)
• at 6 to 8 months of age, mice exhibit decreased spontaneous movement compared to wild-type mice (J:69178)
• at 6 to 8 months of age, mice exhibit decreased spontaneous movement compared to wild-type mice (J:69178)
• eventually mice develop hindlimb paralysis (J:69178)
• eventually mice develop hindlimb paralysis (J:69178)

muscle
• at 6 to 8 months of age, mice exhibit muscle wasting particularly along the flanks (J:69178)
• at 6 to 8 months of age, mice exhibit muscle wasting particularly along the flanks (J:69178)
• mice exhibit spontaneous, positive sharp waves associated with denervation atrophy (J:69178)
• mice exhibit spontaneous, positive sharp waves associated with denervation atrophy (J:69178)

cellular
• motor neurons exhibit mitochondrial degradation (J:69178)
• motor neurons exhibit mitochondrial degradation (J:69178)

growth/size/body
• progressive (J:69178)
• progressive (J:69178)

integument
• at 6 to 8 months of age (J:69178)
• at 6 to 8 months of age (J:69178)

Mouse Models of Human Disease
OMIM ID Ref(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:69178 , J:119631


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
01/26/2016
MGI 6.02
The Jackson Laboratory