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Vol. 15, Issue 10, 4597-4608, October 2004

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Defective Epidermal Barrier in Neonatal Mice Lacking the C-Terminal Region of Connexin43

Karen Maass ^*, Alexander Ghanem , Jung-Sun Kim , Manuela Saathoff , Stephanie Urschel ^*, Gregor Kirfel , Ruth Grümmer ^||, Markus Kretz ^*, Thorsten Lewalter , Klaus Tiemann , Elke Winterhager ^||, Volker Herzog , and Klaus Willecke ^* ¶

^* Institut für Genetik, Universita Bonn, D-53117 Bonn, Germany; Medizinische Klinik und Poliklinik II, Kardiologie und Pneumologie, Universita Bonn, D-53105 Bonn, Germany; University of Ulsan, College of Medicine, Seoul, Republic of Korea; Institut für Zellbiologie, Universita Bonn, D-53121 Bonn, Germany; and ^|| Medizinische Fakulta der Universita Duisburg-Essen, D-45122 Essen, Germany

Submitted April 20, 2004; Revised July 9, 2004; Accepted July 13, 2004
Monitoring Editor: Daniel Goodenough

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.

Abbreviations used: Cx, connexin; HE, hematoxytin & eosin, HO, homozygous; HT, heterozygous; GJIC, gap junction intercellular communication; HPRT, hypoxanthine-guanine phosphoribosyl transferase; PGK, phosphoglycerate kinase; QT_c, heart frequency corrected QT-interval, s., stratum, SEM, scanning electron microscopy, TEM, transmission electron microscopy; WT, wild-type.

The online version of this article contains supplemental material accessible through http://www.molbiolcell.org.

^¶ Corresponding author. E-mail address: genetik{at}uni-bonn.de.

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