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- W3006101815 abstract "Abstract A range of severe human diseases called ciliopathies are caused by the dysfunction of primary cilia. Primary cilia are cytoplasmic protrusions consisting of the basal body (BB), the axoneme and the transition zone (TZ). The BB is a modified mother centriole from which the axoneme, the microtubule-based ciliary scaffold, is formed. At the proximal end of the axoneme, the TZ functions as the ciliary gate governing ciliary protein entry and exit. Since ciliopathies often develop due to mutations in genes encoding proteins that localise to the TZ, the understanding of the mechanisms underlying TZ function is of eminent importance. Here, we show that the ciliopathy protein Rpgrip1l governs ciliary gating by ensuring the proper amount of Cep290 at the vertebrate TZ. Further, we identified the flavonoid eupatilin as a potential agent to tackle ciliopathies caused by mutations in RPGRIP1L as it rescues ciliary gating in the absence of Rpgrip1l." @default.
- W3006101815 created "2020-02-24" @default.
- W3006101815 creator A5005439588 @default.
- W3006101815 creator A5054477830 @default.
- W3006101815 creator A5084109955 @default.
- W3006101815 creator A5086343995 @default.
- W3006101815 creator A5088892803 @default.
- W3006101815 date "2020-02-12" @default.
- W3006101815 modified "2023-09-25" @default.
- W3006101815 title "Rpgrip1l controls ciliary gating by ensuring the proper amount of Cep290 at the vertebrate transition zone" @default.
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