Phenotypes Associated with This Genotype

Genotype
MGI:5493308

• Allelic Composition: Tg(Prnp-CCS)17Jlel/?; Tg(SOD1*G93A)^dl1Gur/?
• Genetic Background: involves: C57BL/6 * SJL

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

mortality/aging

premature death ( J:120361 )

• mean survival age is 36 days

growth/size/body

abnormal postnatal growth ( J:120361 )

• normal growth for the first five days after birth but weight is significantly reduced relative to controls at 6 days

behavior/neurological

tremors ( J:120361 )

• no neurological phenotype through 7 days of age but tremors appear around day 11

ataxia ( J:120361 )

• around day 11

decreased grip strength ( J:120361 )

• marked weakness and declining forelimb strength

short stride length ( J:120361 )

• shorter stride relative to controls at days 13-15

• maximum stride length at 20 days of age and becoming shorter thereafter

muscle

muscle hypertonia ( J:120361 )

• spasticity with extensor hindlimb posturing appears around day 11

nervous system

nervous system phenotype ( J:120361 )

N • no vacuolation in dorsal root ganglia neurons

abnormal hypoglossal nucleus morphology ( J:120361 )

• neuron vacuolation

abnormal motor neuron morphology ( J:120361 )

• vacuoles found in spinal motor neurons at day seven and throughout the brainstem in certain nerves

• dilated vacuoles in lumbar spinal neurons by day 22