Phenotypes Associated with This Genotype

Genotype
MGI:4831000

cx21

• Allelic Composition: Sod2^tm1Cje/Sod2⁺; Tg(SOD1*G93A)1Gur/0
• Genetic Background: involves: C57BL/6 * CD-1 * SJL

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

mortality/aging

premature death ( J:62381 )

• accelerated disease progression

• shorter lifespans than Tg(SOD1*G93A)1Gur mice

• mean decrease in survival is 11 days

behavior/neurological

impaired coordination ( J:62381 )

• worse motor performance in rotorod test than Tg(SOD1*G93A)1Gur mice starting from 106 days of age

nervous system

decreased substantia nigra size ( J:62381 )

• decreased number of total neurons within the substatia nigra at 110 days of age

abnormal motor neuron morphology ( J:62381 )

• microvesiculation within large ventral horn motor neurons at 90 days of age

decreased neuron number ( J:62381 )

• accelerated disease progression

• decreased number of total neurons within the ventral horns of the lumbar cord at 90 days of age

• greater neuronal loss than that in Tg(SOD1*G93A)1Gur mice at 110 days of age

decreased spinal cord ventral horn cell number ( J:62381 )

• decreased number of total neurons within the ventral horns of the lumbar cord starting at 90 days of age

• greater neuronal loss than that in Tg(SOD1*G93A)1Gur mice at 110 days of age

decreased spinal cord size ( J:62381 )

• atrophy of the lumbar spinal cord starting at 90 days of age