Steinberg Gregory R, Carpentier Andre C, Wang Dongdong
Centre for Metabolism, Obesity and Diabetes Research, Division of Endocrinology and Metabolism, Department of Medicine, Faculty of Health Sciences, McMaster University, Hamilton, Ontario, Canada.
Centre de recherche du Centre hospitalier universitaire de Sherbrooke, Department of Medicine, Université de Sherbrooke, Sherbrooke, Quebec, Canada.
J Clin Invest. 2025 Sep 16;135(18). doi: 10.1172/JCI186420.
Metabolic dysfunction-associated steatohepatitis (MASH) is a progressive form of liver disease characterized by hepatocyte injury, inflammation, and fibrosis. The transition from metabolic dysfunction-associated steatotic liver disease (MASLD) to MASH is driven by the accumulation of toxic lipid and metabolic intermediates resulting from increased hepatic uptake of fatty acids, elevated de novo lipogenesis, and impaired mitochondrial oxidation. These changes promote hepatocyte stress and cell death, activate macrophages, and induce a fibrogenic phenotype in hepatic stellate cells (HSCs). Key metabolites, including saturated fatty acids, free cholesterol, ceramides, lactate, and succinate, act as paracrine signals that reinforce inflammatory and fibrotic responses across multiple liver cell types. Crosstalk between hepatocytes, macrophages, and HSCs, along with spatial shifts in mitochondrial activity, creates a feed-forward cycle of immune activation and tissue remodeling. Systemic inputs, such as insulin-resistant adipose tissue and impaired clearance of dietary lipids and branched-chain amino acids, further contribute to liver injury. Together, these pathways establish a metabolically driven network linking nutrient excess to chronic liver inflammation and fibrosis. This Review outlines how coordinated disruptions in lipid metabolism and intercellular signaling drive MASH pathogenesis and provides a framework for understanding disease progression across tissue and cellular compartments.
代谢功能障碍相关脂肪性肝炎(MASH)是一种进行性肝病,其特征为肝细胞损伤、炎症和纤维化。从代谢功能障碍相关脂肪性肝病(MASLD)转变为MASH是由肝脏脂肪酸摄取增加、从头脂肪生成增加以及线粒体氧化受损导致的有毒脂质和代谢中间体的积累所驱动的。这些变化促进肝细胞应激和细胞死亡,激活巨噬细胞,并在肝星状细胞(HSC)中诱导纤维化表型。关键代谢物,包括饱和脂肪酸、游离胆固醇、神经酰胺、乳酸和琥珀酸,作为旁分泌信号,增强多种肝细胞类型的炎症和纤维化反应。肝细胞、巨噬细胞和HSC之间的串扰,以及线粒体活性的空间变化,形成了免疫激活和组织重塑的前馈循环。全身输入,如胰岛素抵抗的脂肪组织以及饮食脂质和支链氨基酸清除受损,进一步加重肝损伤。这些途径共同建立了一个代谢驱动的网络,将营养过剩与慢性肝脏炎症和纤维化联系起来。本综述概述了脂质代谢和细胞间信号传导的协同破坏如何驱动MASH发病机制,并提供了一个理解跨组织和细胞区室疾病进展的框架。