Department of Molecular and Medical Genetics, Indiana University School of Medicine, Indianapolis, Indiana.
Department of Biochemistry and Molecular Biology, Indiana University School of Medicine, Indianapolis, Indiana.
Am J Pathol. 2023 Oct;193(10):1363-1376. doi: 10.1016/j.ajpath.2023.06.006. Epub 2023 Jul 7.
Stress response pathways are crucial for cells to adapt to physiological and pathologic conditions. Increased transcription and translation in response to stimuli place a strain on the cell, necessitating increased amino acid supply, protein production and folding, and disposal of misfolded proteins. Stress response pathways, such as the unfolded protein response (UPR) and the integrated stress response (ISR), allow cells to adapt to stress and restore homeostasis; however, their role and regulation in pathologic conditions, such as hepatic fibrogenesis, are unclear. Liver injury promotes fibrogenesis through activation of hepatic stellate cells (HSCs), which produce and secrete fibrogenic proteins to promote tissue repair. This process is exacerbated in chronic liver disease, leading to fibrosis and, if unchecked, cirrhosis. Fibrogenic HSCs exhibit activation of both the UPR and ISR, due in part to increased transcriptional and translational demands, and these stress responses play important roles in fibrogenesis. Targeting these pathways to limit fibrogenesis or promote HSC apoptosis is a potential antifibrotic strategy, but it is limited by our lack of mechanistic understanding of how the UPR and ISR regulate HSC activation and fibrogenesis. This article explores the role of the UPR and ISR in the progression of fibrogenesis, and highlights areas that require further investigation to better understand how the UPR and ISR can be targeted to limit hepatic fibrosis progression.
应激反应途径对于细胞适应生理和病理条件至关重要。细胞对刺激的转录和翻译增加会对其造成压力,从而需要增加氨基酸供应、蛋白质生成和折叠,以及处理错误折叠的蛋白质。应激反应途径,如未折叠蛋白反应 (UPR) 和综合应激反应 (ISR),允许细胞适应应激并恢复内稳态;然而,它们在病理条件下的作用和调节,如肝纤维化,尚不清楚。肝损伤通过激活肝星状细胞 (HSCs) 促进纤维化,HSCs 产生和分泌纤维生成蛋白以促进组织修复。在慢性肝病中,这个过程会加剧,导致纤维化,如果不加以控制,还会导致肝硬化。纤维生成的 HSCs 表现出 UPR 和 ISR 的激活,部分原因是转录和翻译需求的增加,这些应激反应在纤维化中发挥重要作用。针对这些途径以限制纤维化或促进 HSC 凋亡是一种潜在的抗纤维化策略,但由于我们对 UPR 和 ISR 如何调节 HSC 激活和纤维化的机制理解有限,该策略受到限制。本文探讨了 UPR 和 ISR 在纤维化进展中的作用,并强调了需要进一步研究的领域,以更好地理解如何针对 UPR 和 ISR 来限制肝纤维化进展。