Abstract
article free to read on journal website Although the signaling pathways downstream of receptors such as cytokine receptors are quite well understood, the exact mechanisms employed by many other transmembrane proteins are yet to be fully elucidated. Autosomal dominant polycystic kidney disease, characterized by cysts in the kidney and other organs, is caused by mutations in genes that encode two unrelated and quite distinct transmembrane proteins, polycystin-1 and 2. Polycystin-1 is a large (4302 amino acids) glycoprotein of unknown function, whereas polycystin-2 is related to TRP-like superfamily of channel proteins. The cytoplasmic regions of these two proteins do not have any catalytic activity but are known to interact with each other via coiled-coiled domains. Mice lacking the genes encoding either of these proteins show cyst formation in the kidneys, suggesting that it is possible that their signaling pathways converge...
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