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The proposed schematic of FXN-mediated hBMVEC permeability. (A) Healthy hBMVEC maintain FXN processing, have healthy mitochondrial dynamics, and proper actin polymerization to maintain paracellular tight junctions that prevent paracellular flux. (B) FRDA hBMVECs lose FXN transcriptional processing, lack healthy mitochondrial physiology, and have less polymerized actin at the membrane, leading to unsupported tight junctions and paracellular permeability.
