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Cilia-associated wound repair mediated by IFT88 in retinal pigment epithelium.

TitleCilia-associated wound repair mediated by IFT88 in retinal pigment epithelium.
Publication TypeJournal Article
Year of Publication2023
AuthorsNing, Ke, Bhuckory Mohajeet B., Lo Chien-Hui, Sendayen Brent E., Kowal Tia J., Chen Ming, Bansal Ruchi, Chang Kun-Che, Vollrath Douglas, Berbari Nicolas F., Mahajan Vinit B., Hu Yang, and Sun Yang
JournalSci Rep
Date Published2023 May 21
KeywordsAnimals, Cilia, Ciliopathies, Disease Models, Animal, Humans, Mice, Microtubule-Associated Proteins, Retinal Degeneration, Retinal Pigment Epithelium, Tumor Suppressor Proteins

Primary cilia are conserved organelles that integrate extracellular cues into intracellular signals and are critical for diverse processes, including cellular development and repair responses. Deficits in ciliary function cause multisystemic human diseases known as ciliopathies. In the eye, atrophy of the retinal pigment epithelium (RPE) is a common feature of many ciliopathies. However, the roles of RPE cilia in vivo remain poorly understood. In this study, we first found that mouse RPE cells only transiently form primary cilia. We then examined the RPE in the mouse model of Bardet-Biedl Syndrome 4 (BBS4), a ciliopathy associated with retinal degeneration in humans, and found that ciliation in BBS4 mutant RPE cells is disrupted early during development. Next, using a laser-induced injury model in vivo, we found that primary cilia in RPE reassemble in response to laser injury during RPE wound healing and then rapidly disassemble after the repair is completed. Finally, we demonstrated that RPE-specific depletion of primary cilia in a conditional mouse model of cilia loss promoted wound healing and enhanced cell proliferation. In summary, our data suggest that RPE cilia contribute to both retinal development and repair and provide insights into potential therapeutic targets for more common RPE degenerative diseases.

Alternate JournalSci Rep
PubMed ID37211572
PubMed Central IDPMC10200793
Grant ListP30 EY026877 / EY / NEI NIH HHS / United States
R01 DK114008 / DK / NIDDK NIH HHS / United States
R01 EY024932 / EY / NEI NIH HHS / United States
R01 EY032518 / EY / NEI NIH HHS / United States
R01 EY023295 / EY / NEI NIH HHS / United States