Nephrology Division, Massachusetts General Hospital, Charlestown, MA 02129, USA.
Development. 2011 Aug;138(16):3387-97. doi: 10.1242/dev.066340. Epub 2011 Jul 13.
Intracellular Ca²⁺ signals influence gastrulation, neurogenesis and organogenesis through pathways that are still being defined. One potential Ca²⁺ mediator of many of these morphogenic processes is CaMK-II, a conserved calmodulin-dependent protein kinase. Prolonged Ca²⁺ stimulation converts CaMK-II into an activated state that, in the zebrafish, is detected in the forebrain, ear and kidney. Autosomal dominant polycystic kidney disease has been linked to mutations in the Ca²⁺-conducting TRP family member PKD2, the suppression of which in vertebrate model organisms results in kidney cysts. Both PKD2-deficient and CaMK-II-deficient zebrafish embryos fail to form pronephric ducts properly, and exhibit anterior cysts and destabilized cloacal cilia. PKD2 suppression inactivates CaMK-II in pronephric cells and cilia, whereas constitutively active CaMK-II restores pronephric duct formation in pkd2 morphants. PKD2 and CaMK-II deficiencies are synergistic, supporting their existence in the same genetic pathway. We conclude that CaMK-II is a crucial effector of PKD2 Ca²⁺ that both promotes morphogenesis of the pronephric kidney and stabilizes primary cloacal cilia.
细胞内 Ca²⁺信号通过仍在定义中的途径影响原肠胚形成、神经发生和器官发生。CaMK-II 是一种保守的钙调蛋白依赖性蛋白激酶,可能是许多形态发生过程中的一种潜在 Ca²⁺介质。Ca²⁺的持续刺激将 CaMK-II 转化为激活状态,在斑马鱼中,在前脑、耳朵和肾脏中检测到这种状态。常染色体显性多囊肾病与钙通道 TRP 家族成员 PKD2 的突变有关,在脊椎动物模型生物中抑制 PKD2 会导致肾脏囊肿。PKD2 缺陷型和 CaMK-II 缺陷型斑马鱼胚胎均不能正常形成前肾管,并且表现出前侧囊肿和不稳定的泄殖腔纤毛。PKD2 的抑制在前肾细胞和纤毛中使 CaMK-II 失活,而组成性激活的 CaMK-II 则可恢复 pkd2 形态发生体中的前肾管形成。PKD2 和 CaMK-II 的缺陷具有协同作用,支持它们存在于相同的遗传途径中。我们的结论是,CaMK-II 是 PKD2 Ca²⁺的关键效应因子,它既能促进前肾的形态发生,又能稳定初级泄殖腔纤毛。