Chami Mounia, Checler Frédéric
Université de Nice Sophia Antipolis, IPMC, Sophia Antipolis, F-06560, France.
CNRS, IPMC, Sophia Antipolis, F-06560, France.
Curr Alzheimer Res. 2020;17(4):313-323. doi: 10.2174/1567205017666200225102941.
Pathologic calcium (Ca2+) signaling linked to Alzheimer's Disease (AD) involves the intracellular Ca2+ release channels/ryanodine receptors (RyRs). RyRs are macromolecular complexes where the protein-protein interactions between RyRs and several regulatory proteins impact the channel function. Pharmacological and genetic approaches link the destabilization of RyRs macromolecular complexes to several human pathologies including brain disorders. In this review, we discuss our recent data, which demonstrated that enhanced neuronal RyR2-mediated Ca2+ leak in AD is associated with posttranslational modifications (hyperphosphorylation, oxidation, and nitrosylation) leading to RyR2 macromolecular complex remodeling, and dissociation of the stabilizing protein Calstabin2 from the channel. We describe RyR macromolecular complex structure and discuss the molecular mechanisms and signaling cascade underlying neuronal RyR2 remodeling in AD. We provide evidence linking RyR2 dysfunction with β-adrenergic signaling cascade that is altered in AD. RyR2 remodeling in AD leads to histopathological lesions, alteration of synaptic plasticity, learning and memory deficits. Targeting RyR macromolecular complex remodeling should be considered as a new therapeutic window to treat/or prevent AD setting and/or progression.
与阿尔茨海默病(AD)相关的病理性钙(Ca2+)信号传导涉及细胞内Ca2+释放通道/兰尼碱受体(RyRs)。RyRs是大分子复合物,其中RyRs与几种调节蛋白之间的蛋白质-蛋白质相互作用会影响通道功能。药理学和遗传学方法将RyRs大分子复合物的不稳定与包括脑部疾病在内的多种人类病理联系起来。在本综述中,我们讨论了我们最近的数据,这些数据表明AD中增强的神经元RyR2介导的Ca2+泄漏与翻译后修饰(过度磷酸化、氧化和亚硝基化)有关,这些修饰导致RyR2大分子复合物重塑,以及稳定蛋白钙调蛋白2从通道解离。我们描述了RyR大分子复合物的结构,并讨论了AD中神经元RyR2重塑的分子机制和信号级联反应。我们提供了将RyR2功能障碍与AD中改变的β-肾上腺素能信号级联联系起来的证据。AD中的RyR2重塑会导致组织病理学损伤、突触可塑性改变、学习和记忆缺陷。靶向RyR大分子复合物重塑应被视为治疗/预防AD发生和/或进展的新治疗窗口。