X-linked dominant Charcot-Marie-Tooth disease and other potential gap-junction diseases of the nervous system

David C. Spray, Rolf Dermietzel

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89 Scopus citations


Gap junctions play important roles in the exchange of information and metabolites in the nervous system. These roles are highlighted by a peripheral neuropathy (X-linked dominant Charcot-Marie-Tooth disease) that is associated with mutations in a gap-junction protein (connexin32), resulting in loss of function, and by somatic dysfunctions where changes in expression, organization or function of gap junctions are associated with neuronal hyper- or hypoexcitability. In this review, the causes and consequences of this gap-junction-related peripheral neuropathy and other pathological conditions of the nervous system, where dysfunctions of junctional communication are considered to play a causal role, are considered.

Original languageEnglish (US)
Pages (from-to)256-262
Number of pages7
JournalTrends in Neurosciences
Issue number6
Publication statusPublished - 1995


ASJC Scopus subject areas

  • Neuroscience(all)

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