Abstract
Recessive dystrophic epidermolysis bullosa (RDEB) is a rare skin disorder characterized by defects in collagen VII leading to a range of pathologies dominated by painful blister formation. Aberrant wound healing leads to fibrosis that contributes to life threatening cancers. We have developed an in vitro model of fibrotic extracellular matrix (ECM) produced by primary RDEB patient dermal fibroblasts, in which we use detachment of the cells’ matrix from plastic as a surrogate for fibrosis. This study first sought to determine the molecular mechanisms of matrix detachment, using Atomic Force Microscopy, total collagen assays, and measurements of gene and protein expression, and then assessed application of chemical compounds to reduce fibrosis.
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