ABSTRACT
Fibrillar collagens are widely abundant proteins critical for proper extracellular matrix (ECM) function across many healthy tissues. It follows that abnormal collagen remodeling is not surprisingly linked to a host of disease conditions, including cardiac fibrosis, pulmonary fibrosis, kidney fibrosis, liver fibrosis, tendon scarring, scleroderma, cystic fibrosis, rheumatoid arthritis, and even cancer metastasis (Wynn 2008; Wynn and Ramalingam 2012; Zeisberg and Kalluri 2013). Generally, fibrosis is characterized by ECM accumulation in response to increased matrix production or decreased protease activity, and typically develops following injury and/or inflammation. In most tissues, the primary cell type contributing to matrix buildup is the fibroblast, especially in its activated myo-fibroblast state.
