Ciliopathy-associated IFT144/WDR19 variants differentially influence osteogenic differentiation in mesenchymal stem cells

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ID: 339541
2026
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Abstract
Primary cilia play crucial roles in embryonic development by sensing and transducing extracellular signals, including those from the Hedgehog pathway. Abnormal bone formation is a prominent feature of the ciliopathies, as differentiation of mesenchymal stem cells (MSCs) into osteoblasts involves Hedgehog signaling. We here investigated the association between defects in cilia and defects in osteogenic differentiation resulting from skeletal ciliopathy variants of IFT144/WDR19, a subunit of the IFT-A complex involved in the import into cilia of membrane proteins and in their retrograde trafficking and export. To this end, we established Ift144-knockout cells from the mouse MSC-like C3H10T1/2 cell line and analyzed the phenotypes of these cells expressing IFT144 variants. Ift144-knockout cells expressing the skeletal ciliopathy IFT144 variants were impaired in their differentiation into alkaline phosphatase-positive cells upon Hedgehog pathway activation. These cells also demonstrated defects in the import and export of the Hedgehog pathway GPCRs, Smoothened and GPR161, and in expression of the GLI1 transcription factor that plays a pivotal role in Hedgehog signaling. These results suggest that skeletal ciliopathy IFT144 variants impair osteogenic differentiation, at least in part by impairing Hedgehog signaling resulting from defects in the ciliary localization of Smoothened and GPR161 and in the expression of Gli1.
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Authors Yuto Takahashi, Yamato Ishida, Yohei Katoh, Hye‐Won Shin, Kazuhisa Nakayama
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Journal The Journal of Biochemistry
Year 2026
DOI
10.1093/jb/mvag084
URL
https://doi.org/10.1093/jb/mvag084
Keywords Keywords not found

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