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

Overview

Journal Mol Biol Cell

Specialties Cell Biology
Molecular Biology

Date 2004 Jul 30

PMID 15282340

Citations 67

Authors

Karen Maass

Alexander Ghanem

Jung-Sun Kim

Manuela Saathoff

Stephanie Urschel

Gregor Kirfel

Ruth Grummer

Markus Kretz

Thorsten Lewalter

Klaus Tiemann

Elke Winterhager

Volker Herzog

Klaus Willecke

Affiliations

Soon will be listed here.

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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