Defective epidermal barrier in neonatal mice lacking the C-terminal region of connexin43 |
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Authors: | Maass Karen Ghanem Alexander Kim Jung-Sun Saathoff Manuela Urschel Stephanie Kirfel Gregor Grümmer Ruth Kretz Markus Lewalter Thorsten Tiemann Klaus Winterhager Elke Herzog Volker Willecke Klaus |
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Institution: | Institut für Genetik, Universitat Bonn, D-53117 Bonn, Germany. |
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Abstract: | More than 97% of mice in which the C-terminal region of connexin43 (Cx43) was removed (designated as Cx43K258stop) die shortly after birth due to a defect of the epidermal barrier. The abnormal expression of Cx43K258stop protein in the uppermost layers of the epidermis seems to perturb terminal differentiation of keratinocytes. In contrast to Cx43-deficient mice, neonatal Cx43K258stop hearts show no lethal obstruction of the right ventricular outflow tract, but signs of dilatation. Electrocardiographies of neonatal hearts reveal repolarization abnormalities in 20% of homozygous Cx43K258stop animals. The very rare adult Cx43K258stop mice show a compensation of the epidermal barrier defect but persisting impairment of cardiac function in echocardiography. Female Cx43K258stop mice are infertile due to impaired folliculogenesis. Our results indicate that the C-terminally truncated Cx43K258stop mice lack essential functions of Cx43, although the truncated Cx43 protein can form open gap junctional channels. |
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