Fig. 4.

Limb phenotypes. Control mice from both colonies (HCN and LCN) maintained normal hindlimb and forelimb functions (a). All HCN-SOD1 transgenic mice developed bilateral hindlimb paralysis (b), whereas LCN-SOD1 transgenic mice displayed a variety of limb phenotypes ranging from bilateral hindlimb paralysis (b) to bilateral forelimb paralysis (c), and combined hindlimb and forelimb paralysis affecting one or both sides (d). Note the bedding on the face of the forelimb-affected mouse (c), which demonstrates reduced grooming ability. Also note the lateral forelimb posture during ambulation (d), demonstrating altered weight-bearing capacity of the affected forelimb. As expected, both SOD1 strains displayed a variety of coat colors due to the mixed background strains (i.e., C57BL/6 and SJL) that result in random segregation of coat color alleles. Possible coat colors include white bellied agouti, albino, black, light gray, and tan, some of which are depicted here