Mouse Genome Informatics
tg
    Tg(SOD1*G37R)42Dpr/0
involves: C3H/HeJ * C57BL/6J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
nervous system
• in the choroid plexus
• mice exhibit vacuolization in the neutropil of substantia gelatinosa and scattered in some of the fiber tracts of the ventral and lateral white matter of the spinal cord
• 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
• mice exhibit axonal degradation in dorsal roots of motor neurons

behavior/neurological
• at 3.5 to 4 months of age, mice exhibit axial tremors
• at 3.5 to 4 months of age, mice exhibit asymmetric weakness of the limbs and when suspended by their tail exhibit difficulties extending and moving hindlimbs
• at 3.5 to 4 months of age, mice exhibit decreased spontaneous movement compared to wild-type mice
• eventually mice develop hindlimb paralysis

muscle
• at 3.5 to 4 months of age, mice exhibit muscle wasting particularly along the flanks
• mice exhibit spontaneous, positive sharp waves associated with denervation atrophy

cellular
• motor neurons exhibit mitochondrial degradation

growth/size
• progressive

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

integument
• at 3.5 to 4 months of age

Mouse Models of Human Disease
OMIM IDRef(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:69178