Quantitative studies of the abnormal axon-Schwann cell relationship in the peripheral motor and sensory nerves of the dystrophic mouse.

The nature and extent of abnormal axon-Schwann cell relationships in peripheral portions of dystrophic motor and sensory nerves were quantitatively evaluated between 1 and 9 months of age using teased fibres and electron micrographs. The results show that in the dystrophic (dy/dy) common peroneal (CPN) and tibial nerves (TN), and less in the dy/dy sural nerve (SN): (1) the number of Schwann cell nuclei associated with myelinated axons is increased with respect to normal; (2) the average internodal length is correspondingly reduced; (3) the average dystrophic internode elongates roughly in parallel with the average normal internode, and with the dystrophic limb; the longitudinal growth of the dystrophic limb is normal; (4) the variation of internodal length is greater than normal; it does not increase with age; (5) the incidence of the nodes of Ranvier which are wider than the normal 3 micrometers limit does not increase with age; and (6) the number of myelinated axons is reduced in the dy/dy CPN and TN but not in the dy/dy SN; it shows no change with age. These data indicate that: (1) in the dy/dy peripheral nerves (PNS) the abnormal axon-Schwann cell relationships and the reduced number of myelinated axons have been established prior to 1 month of age, thereafter progressive degenerative processes do not appear to take place, and (2) the dy/dy sensory nerves are less affected than the motor ones.