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

• 主要な著者: Ilona Tietzová (著者), Kirk Twaroski (著者), Cindy Eide (著者), Julie H. Ostrander (著者), Peter A. Crawford (著者), Jakub Tolar (著者)
• フォーマット: 図書
• 出版事項: European Medical Journal, 2020-12-01T00:00:00Z.
• 主題: article
• オンライン･アクセス: 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...