Figure 7

EC dysfunction in CTEPH (a proposed mechanism). Our experiments with ECs and MFLCs demonstrated that the microenvironment provided by the thrombus cells in CTEPH patients might induce EC dysfunction through EnMT, inactivation of autophagy, disruption of mitochondrial reticulum, and the improper SOD-2 localization, although it remains unknown whether both EnMT and other cell function alterations are taking place simultaneously in the same ECs. Although it is uncertain whether MFLCs induce EC dysfunction in vivo and whether EC dysfunction contribute to the vascular lesions in the patients with CTEPH, it is possible that there exist dysfunctional ECs in the microenvironment created by the unresolved clot.