Enculescu Mihaela, Metzendorf Christoph, Sparla Richard, Hahnel Maximilian, Bode Johannes, Muckenthaler Martina U, Legewie Stefan
Institute of Molecular Biology, Mainz, Germany.
Pediatric Oncology, Hematology & Immunology, University Hospital Heidelberg, Heidelberg, Germany.
PLoS Comput Biol. 2017 Jan 9;13(1):e1005322. doi: 10.1371/journal.pcbi.1005322. eCollection 2017 Jan.
Systemic iron levels must be maintained in physiological concentrations to prevent diseases associated with iron deficiency or iron overload. A key role in this process plays ferroportin, the only known mammalian transmembrane iron exporter, which releases iron from duodenal enterocytes, hepatocytes, or iron-recycling macrophages into the blood stream. Ferroportin expression is tightly controlled by transcriptional and post-transcriptional mechanisms in response to hypoxia, iron deficiency, heme iron and inflammatory cues by cell-autonomous and systemic mechanisms. At the systemic level, the iron-regulatory hormone hepcidin is released from the liver in response to these cues, binds to ferroportin and triggers its degradation. The relative importance of individual ferroportin control mechanisms and their interplay at the systemic level is incompletely understood. Here, we built a mathematical model of systemic iron regulation. It incorporates the dynamics of organ iron pools as well as regulation by the hepcidin/ferroportin system. We calibrated and validated the model with time-resolved measurements of iron responses in mice challenged with dietary iron overload and/or inflammation. The model demonstrates that inflammation mainly reduces the amount of iron in the blood stream by reducing intracellular ferroportin transcription, and not by hepcidin-dependent ferroportin protein destabilization. In contrast, ferroportin regulation by hepcidin is the predominant mechanism of iron homeostasis in response to changing iron diets for a big range of dietary iron contents. The model further reveals that additional homeostasis mechanisms must be taken into account at very high dietary iron levels, including the saturation of intestinal uptake of nutritional iron and the uptake of circulating, non-transferrin-bound iron, into liver. Taken together, our model quantitatively describes systemic iron metabolism and generated experimentally testable predictions for additional ferroportin-independent homeostasis mechanisms.
必须将全身铁水平维持在生理浓度,以预防与缺铁或铁过载相关的疾病。在这一过程中起关键作用的是铁转运蛋白,它是已知的唯一哺乳动物跨膜铁输出蛋白,可将铁从十二指肠肠上皮细胞、肝细胞或参与铁循环的巨噬细胞释放到血流中。铁转运蛋白的表达受到转录和转录后机制的严格控制,通过细胞自主和全身机制对缺氧、缺铁、血红素铁和炎症信号作出反应。在全身水平上,铁调节激素铁调素会根据这些信号从肝脏释放出来,与铁转运蛋白结合并触发其降解。目前尚不完全了解各个铁转运蛋白控制机制的相对重要性及其在全身水平上的相互作用。在此,我们构建了一个全身铁调节的数学模型。该模型纳入了器官铁池的动态变化以及铁调素/铁转运蛋白系统的调节作用。我们通过对饮食铁过载和/或炎症刺激的小鼠进行铁反应的时间分辨测量,对该模型进行了校准和验证。该模型表明,炎症主要通过减少细胞内铁转运蛋白的转录来降低血流中的铁含量,而不是通过铁调素依赖的铁转运蛋白蛋白不稳定来实现。相比之下,对于大范围的饮食铁含量变化,铁调素对铁转运蛋白的调节是铁稳态的主要机制。该模型还进一步揭示,在饮食铁水平非常高时,必须考虑其他稳态机制,包括营养性铁的肠道吸收饱和以及循环中的非转铁蛋白结合铁进入肝脏的摄取。综上所述,我们的模型定量描述了全身铁代谢,并对其他不依赖铁转运蛋白的稳态机制产生了可通过实验验证的预测。