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Qiuli Fu; Generation of lentoid bodies from patient-specific induced pluripotent stem cells derived from urinary cells. Invest. Ophthalmol. Vis. Sci. 2018;59(9):2608. doi: https://doi.org/.
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Presentation Description :
More than 300 congenital cataract (CC)-linked mutations in vary genes have been discovered during the past few decades. However, the lack of appropriate human derived disease models has made it difficult to understand their pathological mechanisms, which has limited researches on curing the disease. Recent reports showed that patient-specific models using human-induced pluripotent stem cells (hiPSCs) could be ideal disease models not only for pathological mechanism investigation but also for research on new clinical drug targets. Human lens models in vitro were first established, in which lentoid bodies (LBs) with a human lens-like transparent structure were generated from human urinary cell derived iPSCs (UiPSCs) using the “fried egg” method. The generated LBs not only showed lens biological properties but also were large and transparent enough for the analysis of optical characteristics, suggesting that the generation of patient-specific LBs could be used in future investigations of cataractogenesis. Therefore, patient-specific iPSCs from CC patients with known mutations were then generated and differentiated into the relevant LBs, in which the cataract process was mimicked. Biological properties and optical characteristics between LBs from normal UiPSCS and LBs from patient-specific UiPSCs were compared to address the underlying mechanism of CC. In conclusion, the LBs that generated from patient-specific iPSCs not only mimicked the vivo process of cataracts, but also avoided the species differences that annoyed researchers for a long time. Thus, it is an ideal disease model for studies of cataractogenesis.
This is an abstract that was submitted for the 2018 ARVO Annual Meeting, held in Honolulu, Hawaii, April 29 - May 3, 2018.
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