Department of Medicine, Division of Cardiology, David Geffen School of Medicine, Los Angeles, CA.
Department of Integrative Biology and Physiology, University of California, Los Angeles, CA.
Hepatology. 2018 Dec;68(6):2182-2196. doi: 10.1002/hep.30113. Epub 2018 Nov 8.
We report the genetic analysis of a "humanized" hyperlipidemic mouse model for progressive nonalcoholic steatohepatitis (NASH) and fibrosis. Mice carrying transgenes for human apolipoprotein E*3-Leiden and cholesteryl ester transfer protein and fed a "Western" diet were studied on the genetic backgrounds of over 100 inbred mouse strains. The mice developed hepatic inflammation and fibrosis that was highly dependent on genetic background, with vast differences in the degree of fibrosis. Histological analysis showed features characteristic of human NASH, including macrovesicular steatosis, hepatocellular ballooning, inflammatory foci, and pericellular collagen deposition. Time course experiments indicated that while hepatic triglyceride levels increased steadily on the diet, hepatic fibrosis occurred at about 12 weeks. We found that the genetic variation predisposing to NASH and fibrosis differs markedly from that predisposing to simple steatosis, consistent with a multistep model in which distinct genetic factors are involved. Moreover, genome-wide association identified distinct genetic loci contributing to steatosis and NASH. Finally, we used hepatic expression data from the mouse panel and from 68 bariatric surgery patients with normal liver, steatosis, or NASH to identify enriched biological pathways. Conclusion: The pathways showed substantial overlap between our mouse model and the human disease.
我们报告了一种用于进展性非酒精性脂肪性肝炎(NASH)和纤维化的“人源化”高脂血症小鼠模型的遗传分析。携带人载脂蛋白 E*3-Leiden 和胆固醇酯转移蛋白转基因并喂食“西方”饮食的小鼠在 100 多个近交系小鼠背景下进行了研究。这些小鼠发生了肝炎症和纤维化,其高度依赖遗传背景,纤维化程度存在巨大差异。组织学分析显示出具有人类 NASH 特征的特征,包括大泡性脂肪变性、肝细胞气球样变、炎症灶和细胞周胶原沉积。时程实验表明,虽然饮食中肝甘油三酯水平持续升高,但肝纤维化发生在大约 12 周时。我们发现,导致 NASH 和纤维化的遗传变异与导致单纯脂肪变性的遗传变异明显不同,这与涉及不同遗传因素的多步模型一致。此外,全基因组关联鉴定了导致脂肪变性和 NASH 的不同遗传位点。最后,我们使用来自小鼠小组和 68 名接受减肥手术的正常肝脏、脂肪变性或 NASH 患者的肝表达数据来鉴定丰富的生物学途径。结论:该途径在我们的小鼠模型和人类疾病之间存在显著重叠。