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Article

Altered Ion Channels in an Animal Model of Charcot-Marie-Tooth Disease Type IA

9 Feb 2005

Abstract excerpt

How demyelination and remyelination affect the function of myelinated axons is a fundamental aspect of demyelinating diseases. We examined this issue in Trembler-J mice, a genetically authentic model of a dominantly inherited demyelinating neuropathy of humans. The K+ channels Kv1.1 and Kv1.2 channels were often improperly located in the paranodal axon membrane, typically associated with improperly formed...

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PMID: 15703401
DOI: 10.1523/jneurosci.3328-04.2005

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