Abstract
Regulation of Flightless may lead to improved wound outcomes in children suffering from Epidermolysis Bullosa
Zlatko Kopecki
PhD candidate Discipline of Paediatrics, University of Adelaide and Wound Healing Laboratory, Women’s And Children’s Health Research Institute
Epidermolysis Bullosa (EB) is a group of inherited disorders characterized by skin fragility and blistering. EB patients experience detachment of an epidermal skin layer, due to altered expression of proteins involved in mediating skin adhesion and cellular migration. Wound healing in patients suffering from EB posses a major challenge in their survival. The severity of blister expression can range from mild blistering to severe bulla formation, scaring, mutilation and amputation of the limbs in children. Depending on different subtypes of this disease majority of patients do not survive to adulthood.
Our previous results have shown that Flightless (Flii) regulates of wound repair and decreasing Flii expression in skin results in improved wound outcomes. We have now shown that Flii has an important role in adhesion of skin layers and regulation of cellular migration. Modulation of Flii expression affects skin cell adhesion. This research identifies Flii as a potential candidate for development of novel therapies aimed at improving wound repair in disease where Flii expression is increased. Also we have examined the expression of Flii in 30 patients suffering from EB, which illustrated increased Flii expression in certain subtypes of EB including both Junctional and Dystrophic EB. Present management of EB disease is mainly supportive and therapy symptomatic as no specific cure exists. Development of new mechanistic-based therapies which improve the rate and quality of wound healing offers a remarkable opportunity to enhance the quality and prolong the life of children suffering from EB.
