Polycystin-1: Function as a mechanosensor |
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Authors: | Georgia Dalagiorgou Efthimia K Basdra Athanasios G Papavassiliou |
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Institution: | 1. Kidney Genetics Group, Academic Nephrology Unit, Department of Infection and Immunity, University of Sheffield Medical School, Sheffield, UK;2. Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota, USA |
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Abstract: | Polycystin-1 (PC1), encoded by the Pkd1 gene, is a large transmembrane protein whose mutation is involved in autosomal dominant polycystic kidney disease. When expressed, PC1 activates a G-protein signaling pathway that subsequently modulates Ca2+ channels. PC1 is highly expressed in developing tissue and via its C-terminus tail forms a complex with polycystin-2; this complex, found to be located at the primary cilia, seems to act as a mechanosensor that could affect proliferation, differentiation and apoptosis of cells. Also, loss of polycystins correlates with disruption of flow-dependent and steady-state intracellular Ca2+ signaling. Despite the lack of clarity on the role of the polycystins as mechanosensor molecules, a new interest in this PCs/primary cilium complex is providing continuously new insights. In this review, some of the known features of PC1 such as structure, function, signaling pathways involved and its role as a possible therapeutic target are being discussed. |
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