Department of Cellular and Molecular Physiology and.
Department of Cell Biology, Yale University School of Medicine, New Haven, Connecticut, USA.
JCI Insight. 2019 Nov 1;4(21):127709. doi: 10.1172/jci.insight.127709.
Worldwide, over a billion people suffer from chronic liver diseases, which often lead to fibrosis and then cirrhosis. Treatments for fibrosis remain experimental, in part because no unifying mechanism has been identified that initiates liver fibrosis. Necroptosis has been implicated in multiple liver diseases. Here, we report that O-linked β-N-acetylglucosamine (O-GlcNAc) modification protects against hepatocyte necroptosis and initiation of liver fibrosis. Decreased O-GlcNAc levels were seen in patients with alcoholic liver cirrhosis and in mice with ethanol-induced liver injury. Liver-specific O-GlcNAc transferase-KO (OGT-LKO) mice exhibited hepatomegaly and ballooning degeneration at an early age and progressed to liver fibrosis and portal inflammation by 10 weeks of age. OGT-deficient hepatocytes underwent excessive necroptosis and exhibited elevated protein expression levels of receptor-interacting protein kinase 3 (RIPK3) and mixed lineage kinase domain-like (MLKL), which are key mediators of necroptosis. Furthermore, glycosylation of RIPK3 by OGT is associated with reduced RIPK3 protein stability. Taken together, these findings identify OGT as a key suppressor of hepatocyte necroptosis, and OGT-LKO mice may serve as an effective spontaneous genetic model of liver fibrosis.
全球有超过 10 亿人患有慢性肝脏疾病,这些疾病常常导致纤维化,然后发展为肝硬化。纤维化的治疗方法仍然处于实验阶段,部分原因是尚未确定引发肝纤维化的统一机制。坏死性凋亡已被牵涉到多种肝脏疾病中。在这里,我们报告 O-连接β-N-乙酰氨基葡萄糖(O-GlcNAc)修饰可防止肝实质细胞坏死性凋亡和肝纤维化的发生。在酒精性肝硬化患者和乙醇诱导的肝损伤小鼠中,观察到 O-GlcNAc 水平降低。肝特异性 O-GlcNAc 转移酶敲除(OGT-LKO)小鼠在早期表现出肝肿大和气球样变性,并在 10 周龄时进展为肝纤维化和门脉炎症。缺乏 OGT 的肝细胞发生过度坏死性凋亡,并表现出受体相互作用蛋白激酶 3(RIPK3)和混合谱系激酶结构域样(MLKL)的蛋白表达水平升高,这是坏死性凋亡的关键介质。此外,OGT 对 RIPK3 的糖基化与 RIPK3 蛋白稳定性的降低有关。总之,这些发现确定了 OGT 是肝实质细胞坏死性凋亡的关键抑制因子,OGT-LKO 小鼠可能是肝纤维化的有效自发遗传模型。