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. 2020 Feb 4;13:3. doi: 10.3389/fnmol.2020.00003

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Copyright © 2020 Accorsi, Cramer and Girgenrath.

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

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Proposed model for signaling pathway of fibrosis in MDC1A. TGF-beta, through integrin-alphaV-mediated activation, leads to upregulation of extra-/matricellular proteins that feedback to activate integrin-alphaV and further activation of TGF-beta. Chronic signaling leads to myofibroblast differentiation and fibrosis. Intervention with Losartan, Halofuginone, or Angiotensin-(1–7) inhibits TGF-beta-mediated fibrosis. OPN, osteopontin; FN, fibronectin; PN, periostin.