Schwann cell involvement in the neurological lesion of the dystonic mutant mouse. A nerve grafting study.
Schwann cell function in the dystonic mutant mouse was studied by grafting peripheral nerve from normal into affected littermates of a C57/BL (Fa.) dt dystonic mouse colony and vice versa. In a control experiment, only unaffected animals of the colony were used, and nerve isografts were found to be ultrastructurally indistinguishable from normal nerve autografts. In addition, the isografts showed no features of the lymphocytic inflammatory rejection reaction observed in normal nerve allografts, and there was evidence that donor Schwann cells remained viable and were active in all isografts examined. When nerve isografts from affected dystonic mutants were implanted into normal littermate nerves, the normal host axons regenerating through the grafted region acquired degenerative changes characteristic of naturally occurring dystonic peripheral nerve. These changes were not seen in the host axons regenerating either outside the dystonic graft regions, or more distally in the host nerve stumps. When normal nerve isografts were implanted into affected dystonic mutant nerves, the dystonic axons regenerated through the normal graft region and became normally myelinated. It is concluded that an underlying Schwann cell defect may be responsible for the abnormalities of the dystonic mouse peripheral neuropathy.
D000818:Animals; D004421:Dystonia / Q000473:pathology*; D051379:Mice; D008818:Mice, Neurologic Mutants; D008854:Microscopy, Electron; D010525:Peripheral Nerves / Q000637:transplantation* / Q000648:ultrastructure; D012583:Schwann Cells / Q000502:physiology* / Q000648:ultrastructure; D014185:Transplantation, Isogeneic