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Publication : Hypomyelination in the peripheral nervous system of shiverer mice and in shiverer in equilibrium normal chimaera.

First Author  Peterson AC Year  1984
Journal  J Comp Neurol Volume  227
Issue  3 Pages  348-56
PubMed ID  6207210 Mgi Jnum  J:7591
Mgi Id  MGI:56061 Doi  10.1002/cne.902270305
Citation  Peterson AC, et al. (1984) Hypomyelination in the peripheral nervous system of shiverer mice and in shiverer in equilibrium normal chimaera. J Comp Neurol 227(3):348-56
abstractText  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 in equilibrium 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.
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