Matrix Research Group, Department of Pathology and Medical Biology, University Medical Center Groningen, University of Groningen , Groningen , Netherlands.
Front Med (Lausanne). 2015 Sep 3;2:59. doi: 10.3389/fmed.2015.00059. eCollection 2015.
Chronic organ injury leads to fibrosis and eventually organ failure. Fibrosis is characterized by excessive synthesis, remodeling, and contraction of extracellular matrix produced by myofibroblasts. Myofibroblasts are the key cells in the pathophysiology of fibrotic disorders and their differentiation can be triggered by multiple stimuli. To develop anti-fibrotic therapies, it is of paramount importance to understand the molecular basis of the signaling pathways contributing to the activation and maintenance of myofibroblasts. Several signal transduction pathways, such as transforming growth factor (TGF)-β, Wingless/Int (WNT), and more recently yes-associated protein 1 (YAP)/transcriptional coactivator with PDZ-binding motif (TAZ) signaling, have been linked to the pathophysiology of fibrosis. Activation of the TGF-β1-induced SMAD complex results in the upregulation of genes important for myofibroblast function. Similarly, WNT-stabilized β-catenin translocates to the nucleus and initiates transcription of its target genes. YAP and TAZ are two transcriptional co-activators from the Hippo signaling pathway that also rely on nuclear translocation for their functioning. These three signal transduction pathways have little molecular similarity but do share one principle: the cytosolic/nuclear regulation of its transcriptional activators. Past research on these pathways often focused on the isolated cascades without taking other signaling pathways into account. Recent developments show that parts of these pathways converge into an intricate network that governs the activation and maintenance of the myofibroblast phenotype. In this review, we discuss the current understanding on the signal integration between the TGF-β, WNT, and YAP/TAZ pathways in the development of organ fibrosis. Taking a network-wide view on signal transduction will provide a better understanding on the complex and versatile processes that underlie the pathophysiology of fibrotic disorders.
慢性器官损伤导致纤维化,最终导致器官衰竭。纤维化的特征是肌成纤维细胞过度合成、重塑和收缩细胞外基质。肌成纤维细胞是纤维化疾病病理生理学中的关键细胞,其分化可被多种刺激触发。为了开发抗纤维化疗法,了解导致肌成纤维细胞激活和维持的信号通路的分子基础至关重要。几种信号转导通路,如转化生长因子 (TGF)-β、Wingless/Int (WNT),以及最近的 yes 相关蛋白 1 (YAP)/含 PDZ 结合基序的转录共激活因子 (TAZ) 信号通路,与纤维化的病理生理学有关。TGF-β1 诱导的 SMAD 复合物的激活导致对肌成纤维细胞功能重要的基因上调。同样,WNT 稳定的 β-连环蛋白易位到细胞核并启动其靶基因的转录。YAP 和 TAZ 是 Hippo 信号通路中的两个转录共激活因子,它们的功能也依赖于核易位。这三个信号转导通路在分子上几乎没有相似性,但有一个共同的原则:其转录激活剂的细胞质/核调节。这些通路的过去研究通常侧重于孤立的级联,而不考虑其他信号通路。最近的发展表明,这些通路的部分相互作用汇聚成一个复杂的网络,该网络控制肌成纤维细胞表型的激活和维持。在这篇综述中,我们讨论了 TGF-β、WNT 和 YAP/TAZ 通路在器官纤维化发展过程中信号整合的最新理解。从网络范围的角度来看信号转导将更好地理解纤维化疾病病理生理学基础的复杂和多样的过程。