![]() The polycystin-2 channel preferentially conducts K + and Na + intraciliary Ca 2+, enhances its open probability. ![]() ![]() Using primary cultures of collecting duct cells, we show that polycystin-2, but not polycystin-1, is a required subunit for the ion channel in the primary cilium. Using a new ADPKD mouse model, we observe primary cilia that are abnormally long in cells associated with cysts after conditional ablation of Pkd1 or Pkd2. This question remains unsolved largely because polycystins localize to the primary cilium – a tiny, antenna-like organelle. ![]() Although a genetic basis of ADPKD is established, we lack a clear understanding of polycystin proteins’ functions as ion channels. Mutations in the polycystin genes, PKD1 or PKD2, results in Autosomal Dominant Polycystic Kidney Disease (ADPKD).
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