Phenotypes Associated with This Genotype

Genotype
MGI:4947055

tg3

• Allelic Composition: Tg(SOD1*G37R)29Dpr/0
• Genetic Background: involves: C3H/HeJ * C57BL/6 * C57BL/6J

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

mortality/aging

premature death ( J:69179 , J:144848 )

• mean life expectancy is 9.5 months (J:69179)

• most of the mice (75%) die before 1 year old (J:69179)

• mean life span is 50.5 +/- 2.6 weeks (J:144848)

• increased mean life span and more surviving neurons after injection of lipophilic iron chelator SIH intraperitoneally (J:144848)

nervous system

motor neuron degeneration ( J:69179 )

• massive axonal loss and cell death in the motor axons in one-year old mice

decreased sensory neuron number ( J:69179 )

• massive axonal loss and cell death in the sensory axons in one-year old mice

growth/size/body

weight loss ( J:144848 )

• body weight drop between 47.5 and 50 weeks of age

• no changes in body weight when treated twice a week with SIH during this period

homeostasis/metabolism

abnormal iron homeostasis ( J:144848 )

• increased mitochondrial ferritin in the ventral horn motor neurons and astrocytes in the spinal cord at 12 months of age

abnormal iron level ( J:144848 )

• iron accumulation (56% increase) in the spinal cord at 12 months of age

• small, round cytoplasmic inclusions in the cell bodies of the large ventral horn motor neurons

• a more diffuse accumulation in the entire cell body in some glial cells in the gray and white matter