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. 2020 Sep 19;21(18):6886. doi: 10.3390/ijms21186886

Figure 6.

Figure 6

Schematic representation of miRNAs, their related gene targets and cellular pathways likely involved in MFS aortopathy. In blue, miRNAs involved with the Hippo signaling pathway (related to adherens junctions) and TP53 signaling (related to cell cycle); in black, miRNAs belonging to the “cell cycle” pathway; in green, miRNAs belonging to the “P53 signaling” pathway. MiR-26a and miR-217 are involved in Hippo signaling, whereas miR-145 and miR-25 are involved with adherens junction regulation, and their downregulation promotes the activation of CDH1/APC genes, allowing for matrix remodeling. MiR-27b, miR-30d, miR-27a and miR-29c are involved in P53 signaling, whereas let-7f-5p is involved in “cell cycle signaling”, likely through CCNA2 and TP53 upregulation. Downregulation of the miR-29 family induces the expression of collagen types I and III, influencing cardiac fibrosis, which is likely responsible for the compromised aortic distensibility and systemic compliance in MFS patients.