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- W964610830 abstract "This chapter focuses on a critical function of oligodendrocytes in long-term axonal support. The principal function of oligodendrocytes is the ensheathment of axons with the subsequent assembly of myelin. The chapter discusses five main topics, which are: axon-glia interaction and myelin assembly, mouse mutants as human myelin disease models, axonal defects in the peripheral nervous system (PNS), axonal defects in central nervous system (CNS) myelin mutants, and an ancestral glial function in axonal support. The role of Schwann cells in the peripheral nervous system is also discussed in detail. With respect to axonal integrity, it is helpful to compare the histopathology of multiple sclerosis (MS) with that of inherited myelin diseases. Several mouse models with mutations in myelin-specific genes have been developed that allows detailed histopathological analyses. The altered expression of peripheral myelin protein of 22 kDa (PMP22), a Schwann cell-specific myelin protein of unknown primary function, results in a variable degree of Schwann cell dysmyelination and demyelination. Whereas the effects of slowed nerve conduction are minimal, the progressive loss of peripheral myelin or another function of PMP-mutant Schwann cells causes axonal abnormalities and, ultimately axonal loss, the basis for muscle weakness and clinical symptoms. These observations indicate that myelin-forming Schwann cells are essential for the long-term survival of axons in the PNS. Axonal defect in PNS, CNS myelin mutants and function of glia in axonal support is highlighted too." @default.
- W964610830 created "2016-06-24" @default.
- W964610830 creator A5038465445 @default.
- W964610830 creator A5044498878 @default.
- W964610830 date "2005-01-01" @default.
- W964610830 modified "2023-10-17" @default.
- W964610830 title "Mutations of Myelination-Associated Genes That Affect Axonal Integrity" @default.
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