Experimental Disease Models

The most common models of ALS are based upon the gene mutations associated with fALS linked to SOD-1 mutations. SOD-1-3 convert superoxide radicals to molecular oxygen and hydrogen peroxide. Copper/zinc (Cu/Zn)

SOD-1 is predominantly localized to the cytosol, but may also be associated with the inner mitochondrial membrane; manganese (Mn) SOD-2 is localized to the mitochondria, and SOD-3 is localized extracellularly. Identified fALS-associated mutations in SOD-1 do not cluster around the catalytic site but are distributed throughout the protein. Originally, it was thought that mutations might decrease the enzyme activity of SOD-1; thus, genetically engineered mice that lacked SOD-1 were produced but failed to recapitulate the phenotype of fALS. Transgenic rodents carrying human SOD-1 with fALS mutations G86R, G93A, G37R, or H46R recapitulate essential features of the disease (motor neuron degeneration, muscle atrophy, paralysis) with involvement of the lower and upper motor neurons as well as lifespan being dependent on gene dosage.

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