Ccrk-Mak/Ick signaling is a ciliary transport regulator essential for retinal photoreceptor survival.

ABSTRACT: Primary cilia are microtubule-based sensory organelles whose dysfunction causes ciliopathies in humans. The formation, function, and maintenance of primary cilia depend crucially on intraflagellar transport (IFT); however, the regulatory mechanisms of IFT at ciliary tips are poorly understood. Here, we identified that the ciliopathy kinase Mak is a ciliary tip-localized IFT regulator that cooperatively acts with the ciliopathy kinase Ick, an IFT regulator. Simultaneous disruption of Mak and Ick resulted in loss of photoreceptor ciliary axonemes and severe retinal degeneration. Gene delivery of Ick and pharmacological inhibition of FGF receptors, Ick negative regulators, ameliorated retinal degeneration in Mak ^-/- mice. We also identified that C

SUBMITTER: Chaya T 

PROVIDER: S-EPMC11412320 | biostudies-literature | 2024 Nov

REPOSITORIES: biostudies-literature