IFT139 regulates Hedgehog signaling and cilia structure through ciliary protein localization.

ABSTRACT: The primary cilium, a microtubule-based membrane protrusion, is essential for eukaryotic development and health. Import and export of proteins in and out of the primary cilium relies on intraflagellar transport protein complexes (IFT) IFT-B and IFT-A, in conjunction with their respective motor proteins. Here, using mouse fibroblast cells, we investigated the function of IFT139 (Thm1, TTC21B) in Hedgehog signaling, cilia structure, and ciliary protein localization, as well as the effect of the P209L ciliopathy mutation on cell proliferation and Hedgehog signaling. In cells without IFT139, Ptch1 retains normal localization, Smo and Gli accumulate in the distal tips of cilia with or without pathway activation, while SuFu fails to accumulate in cilia upon pathway activation. We also found that

SUBMITTER: Nishat K 

PROVIDER: S-EPMC12570150 | biostudies-literature | 2025 Oct

REPOSITORIES: biostudies-literature