Impaired Mitochondrial and Metabolic Function of Fibroblasts Derived from Patients with Recessive Dystrophic and Junctional Epidermolysis Bullosa

• Main Authors: Ilona Tietzová (Author), Kirk Twaroski (Author), Cindy Eide (Author), Julie H. Ostrander (Author), Peter A. Crawford (Author), Jakub Tolar (Author)
• Format: Book
• Published: European Medical Journal, 2020-12-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

Background: Recessive dystrophic epidermolysis bullosa (RDEB) and junctional EB (JEB) are inherited disorders characterised by fragility and blistering of epithelial tissues leading to pain, pruritus, and adherent scarring. The severity and chronic nature of the resultant skin wounds significantly r...