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Ohm Society
Connexon, Connexin, Autosomal Recessive Deafness, Inherited Keratoderma Print E-mail

Dr. Kennedy Connexon is a type of protein composed of six subunits that are called connexins. A connexon of one cell is joined to that of an adjacent cell to form an intercellular channel, a little pipe if you will, consisting of 12 connexin subunits. Clusters of intercellular channels are known as a gap junction because of the minute extracellular "gap" that separates the apposed plasma membranes. Each intercellular channel provides an axial channel that interconnects the cytoplasm of the apposed cells directly and permits the passage of ions and other small molecules between adjacent cells.

Connexins are important to intercellular communication. Historically, gap junctions were first seen using the electron microscopy. They appeared to be specialized structures in the plasma membranes of cells in contact with one another. These specialized structures were then shown to consist of cell-to-cell channels. In analyzing the channels from different tissues, the components of these channels were found to differ. These components were named connexins.

Mutations in the connexins are responsible for a diversity of diseases, including some cases of deafness and some skin disorders. At least four connexins are known to be present in the ear. In many populations, mutations of the connexins are the most frequent cause of autosomal recessive deafness. Connexin mutations are also responsible for several forms of inherited keratoderma - a skin disorder characterized by thickened (hyperkeratotic) skin on the palms and soles as well as peripheral neuropathy and cataracts.

The information in this article is not meant to be medical advice.�Treatment for a medical condition should come at the recommendation of your personal physician.

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