Article
Cultured epithelia from junctional epidermolysis bullosa letalis keratinocytes express the main phenotypic characteristics of the disease.
The British journal of dermatology - 1 Feb 1990
Thomas L, Faure M, Cambazard F, Kanitakis J, Verrando P, Ortonne J P, Thivolet J
Abstract excerpt
Keratinocytes from a 1-week-old male infant with junctional epidermolysis bullosa letalis (JEBL) were grown in vitro and then grafted as multi-layered epithelia onto nude mice, to investigate whether the defect in the dermo-epidermal cohesiveness in the disease is of epidermal and not mesodermal origin. In culture, there was a birefringent ring of cells at the edges of the keratinocyte colonies and in places some...
Topics
- Animals
- Cell Differentiation
- Cells, Cultured
- Epidermolysis Bullosa
- Epithelium
- Humans
- Infant, Newborn
- Intercellular Junctions
- Keratinocytes
- Male
- Mice