Fibrosis, or the accumulation of extracellular matrix molecules that make up scar tissue, is a common feature of chronic tissue injury. Pulmonary fibrosis, renal fibrosis, and hepatic cirrhosis are among the more common fibrotic diseases, which in aggregate represent a huge unmet clinical need. New appreciation of the common features of fibrosis that are conserved among tissues has led to a clearer understanding of how epithelial injury provokes dysregulation of cell differentiation, signaling, and protein secretion. At the same time, discovery of tissue-specific features of fibrogenesis, combined with insights about genetic regulation of fibrosis, has laid the groundwork for biomarker discovery and validation, and the rational identification of mechanism-based antifibrotic drugs. Together, these advances herald an era of sustained focus on translating the biology of fibrosis into meaningful improvements in quality and length of life in patients with chronic fibrosing diseases.
"Table 1 lists a selection of antifibrotic drug trials in liver disease. A more extensive listing and description of promising experimental agents can be found elsewhere   . Importantly, biotech and big pharma have become increasingly interested in liver fibrosis and fibrosis of other organs, which represents a novel and profitable, yet unconconquered area in biomedicine. "
"The direct link between inflammation and fibrosis has been well established as well (Wick et al., 2013). Despite many clinical and experimental investigations, effective treatment for fibrosis is still lacking in the clinic (Friedman et al., 2013). FTY720, an S1P analog, effectively inhibits the egress of T and B cells from lymph nodes (Kabashima et al., 2006; Matloubian et al., 2004), thereby reducing the number of antigen-primed/restimulated cells that recirculate to peripheral inflammatory tissues (Brinkmann and Lynch, 2002), and consequently halts inflammation. "
"The example of TGF-β1 hyper-activity in Marfan syndrome demonstrates that inhibition of LTBP binding to the ECM will need to be investigated with care as one possible strategy to prevent TGF-β1 activation in fibrosis. One the ECM side, current strategies aim at generally reducing the crosslink-degree which contributes to both, scar stiffening and binding of LTBP-1; targets are transglutaminases, lysyl oxidases, lysyl oxidase-like enzymes, and lysyl hydroxylases (reviewed in   ). Proteoglycans, in particular heparin/heparan sulfate are other attractive targets in the ECM due to their mediating role between different fibrillar ECM proteins as well as LTBPs   . "
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