Phenotypes Associated with This Genotype

Genotype
MGI:4947056

cx1

• Allelic Composition: Tg(NEFH)200Jpj/0; 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 )

• extended mean longevity by about 6 months, compared with Tg(SOD1*G37R)29Dpr/0 mice

• all the mice are still alive after 1 year

• average lifespan is 15.8 months

behavior/neurological

paralysis ( J:69179 )

• delayed onset of paralysis

• occur shortly before death with a period of about 2 weeks duration

nervous system

motor neuron degeneration ( J:69179 )

• axonal atrophy

• prominent perikaryal neurofilamentous accumulations

• no massive axonal loss and cell death in the motor axons in one-year old mice

decreased sensory neuron number ( J:69179 )

• axonal atrophy

• prominent perikaryal neurofilamentous accumulations

• no massive axonal loss and cell death in the sensory axons in one-year old mice