0873 Modeling junctional epidermolysis bullosa using tissue-engineered skin substitutes

This study explored the use of tissue-engineered skin substitutes (TES) to model junctional epidermolysis bullosa (JEB), a genetic skin disorder. Researchers cultivated keratinocytes and fibroblasts from a JEB patient and produced TES to measure dermal-epidermal junction (DEJ) adhesion strength. TES with JEB keratinocytes showed low DEJ adhesion strength, while TES with healthy keratinocytes exhibited high adhesion strength. Type XVII collagen, crucial for DEJ integrity, was absent in JEB keratinocytes. The study concluded that TES effectively models the JEB phenotype and suggests that targeting keratinocytes could be key in developing gene therapy for JEB.