Phenotypes Associated with This Genotype

Genotype
MGI:3785392

• Allelic Composition: Tg(SOD1*G93A)^dl1Gur/0
• Genetic Background: involves: C57BL/6 * CBA * SJL

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

mortality/aging

premature death ( J:134095 )

• most symptomatic animals die at <300 days of age

behavior/neurological

impaired coordination ( J:134095 )

• inability to perform hanging wire test

muscle

muscle weakness ( J:134095 )

• first sign of disease is forelimb weakness

progressive muscle weakness ( J:134095 )

• the T3 transgene has marginal effects on disease onset and survival in the G1del mutants; onset is 168 to 197 days with end-stage reached at 210-248 days

• animals show forelimb onset of disease

cellular

abnormal mitochondrial morphology ( J:134095 )

• vacuolated mitochondria are sometimes seen in spinal cord of end-stage animals

nervous system

astrocytosis ( J:134095 )