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. 2020 Jun 19;8:469. doi: 10.3389/fcell.2020.00469

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Copyright © 2020 Zhou, Weber, Zhao, Chen, Barber, Grillo, Wills, Wang, Hulleman and Sundstrom.

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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Working model for RPE cells dysfunction and undergoing EMT. RPE cells exhibit a well-differentiated epithelial phenotype, characterized by integrated tight junctions, a high degree of polarity, and proper RPE signature gene expression. Expression of R345W-Fibulin-3 in RPE cells leads to a mesenchymal-like phenotype with cytoskeletal changes, a transcriptional shift, and an increased capacity for migration. Potential mechanisms contributing to EMT in RPE cells include aging and misfolded protein accumulation.