In removing cilia from human pluripotent stem cells, Freedman and his colleagues sought to understand what would happen in their subsequent transformation into tissues and organoids. As it happened, the cilia-free stem cells appeared normal but were unable to fully realize new forms.
“It was surprising to me that, at a certain point after they were turning into tissues, they seemed to break down,” Freedman said. “They struggled to transform into anything sophisticated. I think one lesson from this is that the cilia help get cells through their final stage of development.”
It was first reported in 2000 that PKD could stem from defects in cilia, but the mechanism of damage that causes cysts to form has escaped scientists. By creating cilia-free stem cells that harbor disease, Freedman said, the researchers now have a framework with which to test and compare molecular actions in the cilia.
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