Defective epidermal barrier in neonatal mice lacking the C-terminal region of connexin43.
Mol Biol Cell
; 15(10): 4597-608, 2004 Oct.
Article
em En
| MEDLINE
| ID: mdl-15282340
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.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Conexina 43
/
Epiderme
Limite:
Animals
Idioma:
En
Ano de publicação:
2004
Tipo de documento:
Article