Phenotypes Associated with This Genotype

Genotype
MGI:4838155

cx22

• Allelic Composition: Tg(Myl1-Igf1)1Nros/0; Tg(SOD1*G93A)1Gur/0
• Genetic Background: involves: C57BL/6 * FVB/N * SJL

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

mortality/aging

premature death ( J:95812 )

• delayed the onset and in particular the progression of disease

• increase the survival by about 30 days to a maximal life span of 175 d

muscle

decreased skeletal muscle fiber diameter ( J:95812 )

• decreased muscle fiber diameter at 123 d and 150 d, compared with Tg(Myl1-Igf1)1Nros mice

• attenuated muscle atrophy, compared with Tg(SOD1*G93A)1Gur mice

centrally nucleated skeletal muscle fibers ( J:95812 )

• at all stages of disease, including at paralysis stage (123 d)

abnormal skeletal muscle fiber type ratio ( J:95812 )

• altered fiber type composition at 138 d with a shift toward a fast fiber type

nervous system

astrocytosis ( J:95812 )

• decrease in astroglial activation at paralysis stage (123 d) in the spinal cord, compared with Tg(SOD1*G93A)1Gur mice

decreased motor neuron number ( J:95812 )

• motor neuron loss is less severe at 112 and 123 d, compared with Tg(SOD1*G93A)1Gur mice