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Trends Neurosci. 1995 Jun;18(6):256-62.

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

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Depts of Neuroscience, Albert Einstein College of Medicine, Bronx, NY, USA.


Gap junctions play important roles in the exchange of information and metabolites in the nervous system. These roles are highlighted by 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 casual role, are considered.

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