Sergio S, Spedicato B, Corallo G, Inguscio A, Greco M, Musarò D, Vergara D, Muro A F, De Sabbata G, Soria L R, Pierri N Brunetti, Maffia M
Laboratory of Clinical Proteomic, "V Fazzi" Hospital, Lecce, 73100, Italy.
Laboratory of General and Human Physiology, Department of Experimental Medicine, University of Salento, Lecce, 73100, Italy.
Adv Biol (Weinh). 2025 Mar;9(3):e2400408. doi: 10.1002/adbi.202400408. Epub 2025 Jan 11.
Ammonia a by-product of nitrogen containing molecules is detoxified by liver into non-toxic urea and glutamine. Impaired ammonia detoxification leads to hyperammonemia. Ammonia has a dual role on autophagy, it acts as inducer at low concentrations and as inhibitor at high concentrations. However, little is known about the mechanisms responsible for this switch. Wnt/β-catenin signalling is emerging for its role in the regulation of ammonia metabolizing enzymes and autophagosome synthesis through c-Myc. Here, using Huh7 cell line, we show a modulation in c-Myc expression under different ammonia concentrations. An increase in c-Myc expression and in its transcriptional regulator β-catenin was detected at low concentrations of ammonia, when autophagy is active, whereas these modifications were lost under high ammonia concentrations. These observations were also recapitulated in the livers of spf-ash mice, a model of constitutive hyperammonaemia due to deficiency in ornithine transcarbamylase enzyme. Moreover, c-Myc-mediated activation of autophagy plays a cytoprotective role in cells under ammonia stress conditions as confirmed through the pharmacological inhibition of c-Myc in Huh7 cells treated with low ammonia concentrations. In conclusion, the unravelled role of c-Myc in modulating ammonia induced autophagy opens new landscapes for the development of novel strategies for the treatment of hyperammonemia.
氨是含氮分子的一种副产物,经肝脏解毒转化为无毒的尿素和谷氨酰胺。氨解毒功能受损会导致高氨血症。氨对自噬具有双重作用,低浓度时作为诱导剂,高浓度时作为抑制剂。然而,对于这种转变背后的机制却知之甚少。Wnt/β-连环蛋白信号通路因其在通过c-Myc调节氨代谢酶和自噬体合成中的作用而受到关注。在此,我们利用Huh7细胞系,展示了在不同氨浓度下c-Myc表达的调控情况。在低氨浓度且自噬活跃时,检测到c-Myc及其转录调节因子β-连环蛋白的表达增加,而在高氨浓度下这些变化消失。这些观察结果在spf-ash小鼠肝脏中也得到了验证,spf-ash小鼠是由于鸟氨酸转氨甲酰酶缺乏导致的遗传性高氨血症模型。此外,通过在低氨浓度处理的Huh7细胞中对c-Myc进行药理学抑制证实,c-Myc介导的自噬激活在氨应激条件下对细胞具有细胞保护作用。总之,c-Myc在调节氨诱导的自噬中的作用揭示,为开发治疗高氨血症的新策略开辟了新前景。