Sandebring Anna, Dehvari Nodi, Perez-Manso Monica, Thomas Kelly Jean, Karpilovski Elena, Cookson Mark R, Cowburn Richard F, Cedazo-Mínguez Angel
Karolinska Institutet, Department of NVS, KI-Alzheimer's Disease Research Center, Stockholm, Sweden.
FEBS J. 2009 Sep;276(18):5041-52. doi: 10.1111/j.1742-4658.2009.07201.x. Epub 2009 Aug 3.
Mutations in the E3 ubiquitin ligase parkin cause early-onset, autosomal-recessive juvenile parkinsonism (AJRP), presumably as a result of a lack of function that alters the level, activity, aggregation or localization of its substrates. Recently, we have reported that phospholipase Cgamma1 is a substrate for parkin. In this article, we show that parkin mutants and siRNA parkin knockdown cells possess enhanced levels of phospholipase Cgamma1 phosphorylation, basal phosphoinositide hydrolysis and intracellular Ca2+ concentration. The protein levels of Ca2+-regulated protein kinase Calpha were decreased in AJRP parkin mutant cells. Neomycin and dantrolene both decreased the intracellular Ca2+ levels in parkin mutants in comparison with those seen in wild-type parkin cells, suggesting that the differences were a consequence of altered phospholipase C activity. The protection of wild-type parkin against 6-hydroxydopamine (6OHDA) toxicity was also established in ARJP mutants on pretreatment with dantrolene, implying that a balancing Ca2+ release from ryanodine-sensitive stores decreases the toxic effects of 6OHDA. Our findings suggest that parkin is an important factor for maintaining Ca2+ homeostasis and that parkin deficiency leads to a phospholipase C-dependent increase in intracellular Ca2+ levels, which make cells more vulnerable to neurotoxins, such as 6OHDA.
E3泛素连接酶帕金蛋白(parkin)的突变会导致早发性常染色体隐性青少年帕金森病(AJRP),这可能是由于功能缺失改变了其底物的水平、活性、聚集或定位所致。最近,我们报道了磷脂酶Cγ1是帕金蛋白的一种底物。在本文中,我们表明,帕金蛋白突变体和经小干扰RNA(siRNA)敲低帕金蛋白的细胞中,磷脂酶Cγ1的磷酸化水平、基础磷酸肌醇水解作用以及细胞内钙离子浓度均有所升高。在AJRP帕金蛋白突变体细胞中,钙离子调节蛋白激酶Cα的蛋白质水平降低。与野生型帕金蛋白细胞相比,新霉素和丹曲林均降低了帕金蛋白突变体细胞内的钙离子水平,这表明这些差异是磷脂酶C活性改变的结果。在用丹曲林预处理的ARJP突变体中,也证实了野生型帕金蛋白对6-羟基多巴胺(6OHDA)毒性具有保护作用,这意味着从兰尼碱敏感储存库中平衡释放钙离子可降低6OHDA的毒性作用。我们的研究结果表明,帕金蛋白是维持钙离子稳态的重要因素,帕金蛋白缺乏会导致细胞内钙离子水平因磷脂酶C而升高,从而使细胞更容易受到神经毒素(如6OHDA)的影响。