Hypomyelination in the peripheral nervous system of shiverer mice and in Shiverer ↔ Normal chimaera

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Abstract
In shiverer mice, the P1 component of myelin basic protein (MBP) is deficient in both the central nervous system (CNS) and peripheral nervous system (PNS) but compact myelin is more grossly defective in the CNS. In the PNS, myelin exhibits a normal periodic structure, and although examples of subtle abnormalities of shiverer Schwann cell ultrastructure have been described previously, myelin thickness has been reported as unremarkable when observed by light microscopy. We report, a quantitative investigation of the myelin sheath thickness of shiverer Schwann cells in which a mild but apparently consistent hypomyelination of axons ensheathed by shiverer Schwann cells was observed. This abnormality was expressed both in the peripheral nerves of a homozygous shiverer mouse and in the shiverer Schwann cells populating the mosaic nerves of a mature ↔ shiverer normal mouse chimaera. In addition, multiple interlamellar gaps was found to be a highly consistent feature of shiverer myelin. These observations extend the description of the peripheral nerve defects expressed in shiverer mice and further define these abnormalities as direct consequences of the shiverer Schwann cells' intrinsic genotype. In light of these results, a significant role for P1 in the formation and/or maintenance of normal myelin in the PNS is suggested.