Motoneuron degeneration after facial nerve avulsion is exacerbated in presymptomatic transgenic rats expressing human mutant Cu/Zn superoxide dismutase.

We investigated motoneuron degeneration after proximal nerve injury in presymptomatic transgenic (tg) rats expressing human mutant Cu/Zn superoxide dismutase (SOD1). The right facial nerves of presymptomatic tg rats expressing human H46R or G93A SOD1 and their non-tg littermates were avulsed, and facial nuclei were examined at 2 weeks postoperation. Nissl-stained cell counts revealed that facial motoneuron loss after avulsion was exacerbated in H46R- and G93A-tg rats compared with their non-tg littermates. The loss of motoneurons in G93A-tg rats after avulsion was significantly greater than that in H46R-tg rats. Intense cytoplasmic immunolabeling for SOD1 in injured motoneurons after avulsion was demonstrated in H46R- and G93A-tg rats but not in their littermates. Facial axotomy did not induce significant motoneuron loss nor enhance SOD1 immunoreactivity in these tg rats and non-tg littermates at 2 weeks postoperation, although both axotomy and avulsion elicited intense immunolabeling for activating transcription factor-3, phosphorylated c-Jun, and phosphorylated heat shock protein 27 in injured motoneurons of all these animals. The present data indicate the increased vulnerability of injured motoneurons after avulsion in the presymptomatic mutant SOD1-tg rats.