MicroRNA‐145‐5p regulates fibrotic features of recessive dystrophic epidermolysis bullosa skin fibroblasts

• Published in: British journal of dermatology (1951) Vol. 181; no. 5; pp. 1017 - 1027
• Main Authors: Condorelli, A.G., Logli, E., Cianfarani, F., Teson, M., Diociaiuti, A., El Hachem, M., Zambruno, G., Castiglia, D., Odorisio, T.
• Format: Journal Article
• Language: English
• Published: England Oxford University Press 01.11.2019
• Subjects: Actin; Adolescent; Adult; Biopsy; Cell Movement - drug effects; Cell Movement - genetics; Cell Proliferation - drug effects; Cell Proliferation - genetics; Cells, Cultured; Child; Collagen; Collagen (type VII); Collagen Type VII - genetics; Contraction; Dystrophic epidermolysis bullosa; Epidermolysis bullosa; Epidermolysis Bullosa Dystrophica - genetics; Epidermolysis Bullosa Dystrophica - pathology; Female; Fibroblasts; Fibroblasts - pathology; Fibrosis; Gene expression; Humans; Infant; Infant, Newborn; Jagged-1 Protein - genetics; Jagged1 protein; Kruppel-Like Transcription Factors - genetics; Male; MicroRNAs; MicroRNAs - antagonists & inhibitors; MicroRNAs - metabolism; Middle Aged; miRNA; Muscle contraction; Mutation; Polymerase chain reaction; Primary Cell Culture; Skin - cytology; Skin - pathology; Skin diseases; Smooth muscle; Up-Regulation; Wound healing

Summary Background Recessive dystrophic epidermolysis bullosa (RDEB) is a skin fragility disorder caused by mutations in the COL7A1 gene encoding type VII collagen, a cutaneous basement membrane component essential for epidermal–dermal adhesion. Hallmarks of the disease are unremitting blistering an...