Shiverer mouse

Shiverer mouse, myelin-deficient mouse AR Myelin basic protein (MBP) Deletion or inversion of several MBP exons very little functional MBP expressed severe CNS hypomyelination and failure of compaction of major dense line see text 1,10,43... 

Yandava, B. D., Billinghurst, L. L. and Snyder, E. Y. Global cell replacement is feasible via neural stem cell transplantation evidence from the dysmyelinated shiverer mouse brain. Proc. Natl Acad. Sci. U.S.A. 96 7029-7034,1999. 

Myelin basic proteins (MBP). Basic proteins are abundant both in CNS and PNS myelin, where they are associated with negatively charged lipids. They are assumed to be involved in myelin compaction on the cytoplasmic side of the membrane bilayer. A spontaneous MBP mutant, the shiverer mouse, is devoid of the major dense line of myelin in the CNS (Dupouey et al., 1979). Another such mutant, the Long Evans Shaker (LES) rat, shows major changes in spinal cord white matter, with dispersed labeling of Kvl.l and Kvl.2 K+ channel subunits as well as of Caspr, a molecule normally confined to paranodes along LES rat spinal axons (Eftekharpour et al, 2005). 

Westenbroek RE, Noebels JL, Catterall WA 1992 Elevated expression of type II Na channels in hypomyelinated axons of shiverer mouse brain. J Neurosci 12 2259-2267... 

Noebe/s I think the latter is correct, because myelin basic protein is actually present in oligodendrocytes centrally and Schwann cells peripherally. But in the shiverer mouse, Schwann cell myelination of peripheral nerves is relatively unaffected by the same genetic lesion that prevents formation of the central white matter. The protein must either play a different role in those two cell types, or they signal to axons in different ways. Whether it is there or not, if you irritate a ghal ceh, the oligodendrocyte will say one thing to a central axon and the Schwann cells may say something different to a peripheral axon. 

Ptacek One could synthesize a phosphotyrosine-specific antibody. Then it would be interesting to look at the shiverer mouse, to look for differences between phosphotyrosine versus the non-phosphorylated form. 

SAolecular plasticity of Na channel gene expression the dysmyelinating shiverer mouse... 

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