Figure 7.

Model illustrating the potential pathway from pseudohypoxia-induced cilia loss to increased cell proliferation and dysregulation of tumorigenesis relevant cilia-mediated signaling pathways in PCCs. Proteins where germline mutations in the gene predispose to PCC are in orange boxes. Aurora-A, Aurora-A kinase; FH, fumarate hydratase; H6, HDAC6; HIF, hypoxia-inducible factor; OH, hydroxyl group; P, phosphate group; PH, prolyl-hydroxylases; SDH, succinate dehydrogenase; VHL, von Hippel–Lindau protein.