Phenotypes Associated with This Genotype

Genotype
MGI:3785393

• Allelic Composition: Tg(SOD1)2Gur/0; Tg(Thy1-SOD1*G93A)T3Hgrd/0
• Genetic Background: involves: C57BL/6 * CBA * FVB * SJL

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

nervous system

abnormal oligodendrocyte morphology ( J:134095 )

• ubiquinated SOD1 aggregates are seen at >45 weeks

abnormal innervation pattern to muscle ( J:134095 )

• muscle denervation is seen at end-stage of disease (<80 weeks)

decreased motor neuron number ( J:134095 )

• motor neuron loss characterizes end-stage of disease (<80 weeks)

abnormal dendrite morphology ( J:134095 )

• ubiquinated dendritic SOD1 aggregates develop; these are present as early as 15-20 weeks

behavior/neurological

paralysis ( J:134095 )

• most mice show total paralysis of at least one hind limb; at end-stage, >80% of mice display fully immobilized hindlimbs

muscle

muscle weakness ( J:134095 )

• develops at about 1 year of age

• reach end-stage of disease before 80 weeks

• 60% of animals show hindlimb onset of disease while subset displays forelimb onset with short disease duration

cellular

abnormal oligodendrocyte morphology ( J:134095 )

• ubiquinated SOD1 aggregates are seen at >45 weeks