Phenotypes Associated with This Genotype

Genotype
MGI:4820956

• Allelic Composition: Tg(SOD1*G93A)G20Gur/0
• Genetic Background: involves: C57BL/6 * SJL

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

mortality/aging

premature death ( J:78630 )

• deaths occurred at 332 and 354 days of age due to severe paralysis of all four limbs

nervous system

motor neuron degeneration ( J:78630 )

• at 350 days of age

• atrophy of the anterior horn due to loss of neurons

• filamentous inclusions of varying size and shape in neurons and axons

• mild rarefaction of the neuropil, mild increase in astrocytic nuclei

• rare examples of vacuolar changes, sometimes in surviving neurons, sometimes in dendrites or axons

• only minimal involvement of the posterior columns

abnormal spinal nerve morphology ( J:78630 )

• the largest fibers in brachial and lumbo-sacral plexuses are completely replaced by strings of large lipid laden macrophages

abnormal sciatic nerve morphology ( J:78630 )

• the largest fibers in lumbo-sacral plexuses are completely replaced by strings of large lipid laden macrophages

abnormal ventral spinal root morphology ( J:78630 )

• loss of axons, increased perineurial space and, frequently, numerous myelin debris-laden macrophages

• no involvement of the posterior roots.