Morphometric and freeze-fracture studies on peripheral nerve in shiverer mice

Observations have been made on the peripheral nerves of shiverer (shi/shi) mice in comparison with control animals. Although this mutant lacks P1 myelin basic protein in peripheral and central myelin, myelin is defective only in the central nervous system. No ultrastructural abnormalities were observed in the shiverer nerves. Myelin spacing was normal. The density and distribution of intramembranous particles on the E and P faces of myelin and in the axolemma of myelinated and unmyelinated axons did not differ between the shiverer and control mice. Morphometric studies showed that external myelinated fiber diameter was significantly less and that myelin thickness was slightly but significantly greater in relation to axon diameter in the shiverer mice, suggesting a minor degree of axonal atrophy. It is concluded that P1 protein is not necessary for the formation and maintenance of the normal structure of peripheral myelin. The failure to detect differences in intramembranous particle density in myelin between shiverer and control mice indicates that P1 protein is not detected in freeze-fracture preparations.