Eupatilin Improves Cilia Defects in Human CEP290 Ciliopathy Models
Abstract
:1. Introduction
2. Materials and Methods
2.1. Cell Lines
2.2. qPCR
2.3. Western Blot
2.4. Cell Culture and Eupatilin Treatment
2.5. Immunocytochemistry
2.6. iPSC Differentiation to Retinal Organoids
2.7. Cryopreservation and Immunohistochemistry of Retinal Organoids
2.8. Cilia Quantification and Statistical Analysis
2.9. RNAseq Pre-Processing and Analysis
3. Results
3.1. Eupatilin Increases Cilia Incidence and Length in CEP290 LCA10 Patient Fibroblasts
3.2. Eupatilin Restores Cilia Incidence and Length in CEP290 Knockout RPE1 Cells
3.3. Eupatilin Treatment Restores Ciliation and Cilia Length in iPSC-Derived CEP290 LCA10 and CEP290 KO Retinal Organoids
3.4. Opsin Accumulates in the Outer Nuclear Layer of CEP290 LCA10 Retinal Organoids and Is Rescued by Eupatilin
3.5. Eupatilin Treatment Reduces the Expression of Rhodopsin
3.6. Eupatilin Modulates the Expression of Ciliary and Synaptic Pathways
4. Discussion
Supplementary Materials
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
References
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Corral-Serrano, J.C.; Sladen, P.E.; Ottaviani, D.; Rezek, O.F.; Athanasiou, D.; Jovanovic, K.; van der Spuy, J.; Mansfield, B.C.; Cheetham, M.E. Eupatilin Improves Cilia Defects in Human CEP290 Ciliopathy Models. Cells 2023, 12, 1575. https://doi.org/10.3390/cells12121575
Corral-Serrano JC, Sladen PE, Ottaviani D, Rezek OF, Athanasiou D, Jovanovic K, van der Spuy J, Mansfield BC, Cheetham ME. Eupatilin Improves Cilia Defects in Human CEP290 Ciliopathy Models. Cells. 2023; 12(12):1575. https://doi.org/10.3390/cells12121575
Chicago/Turabian StyleCorral-Serrano, Julio C., Paul E. Sladen, Daniele Ottaviani, Olivia F. Rezek, Dimitra Athanasiou, Katarina Jovanovic, Jacqueline van der Spuy, Brian C. Mansfield, and Michael E. Cheetham. 2023. "Eupatilin Improves Cilia Defects in Human CEP290 Ciliopathy Models" Cells 12, no. 12: 1575. https://doi.org/10.3390/cells12121575