Zusammenfassung
Plakophiline (Plakophilin 1a/b, 2a/b, 3) zählen zu den desmosomalen Plaque-Proteinen. Plakophilin 1 ist in der fetalen Epidermis bereits in der 10. Gestationswoche nachweisbar, und zwar zunächst nur in den suprabasalen, dann in allen epidermalen Schichten. Plakophilin 2 wird hingegen nur in der frühen Fetalzeit in der Basalschicht exprimiert. Eine Mutation des Plakophilin 1 Gens (PKP1) führt zu Akantholyse und Zeichen der ektodermalen Dysplasie. Diese und andere Beobachtungen sprechen dafür, daß den Plakophilinen neben ihrer Strukturfunktion auch eine Bedeutung bei der intrazellulären Signaltransduktion bzw. bei der epidermalen Morphogenese zukommt.
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Höger, P.H., Brandner, J., Finger, G., Moll, I. (2000). Normale fetale Entwicklung und genetische Defekte der Plakophilinexpression beim Menschen. In: Plettenberg, A., Meigel, W.N., Moll, I. (eds) Dermatologie an der Schwelle zum neuen Jahrtausend. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-57191-6_107
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DOI: https://doi.org/10.1007/978-3-642-57191-6_107
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