Departments of Medicine (GI) and Pathology and Laboratory Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
Gastroenterology. 2014 Jul;147(1):41-7. doi: 10.1053/j.gastro.2014.05.001. Epub 2014 May 9.
Portal fibroblasts, the resident fibroblasts of the portal tract, are found in the mesenchyme surrounding the bile ducts. Their roles in liver homeostasis and response to injury are undefined and controversial. Although portal fibroblasts almost certainly give rise to myofibroblasts during the development of biliary fibrosis, recent lineage tracing studies suggest that their contribution to fibrogenesis is limited compared with that of hepatic stellate cells. Other functions of portal fibroblasts include participation in the peribiliary stem cell niche, regulation of cholangiocyte proliferation, and deposition of specific matrix proteins. Portal fibroblasts synthesize elastin and other components of microfibrils; these may serve structural roles, providing stability to ducts and the vasculature under conditions of increased ductal pressure, or could regulate the bioavailability of the fibrogenic transforming growth factor β in response to injury. Viewing portal fibroblasts in the context of fibroblast populations throughout the body and studying their niche-specific roles in matrix deposition and epithelial regulation could yield new insights into their contributions in the normal and injured liver. Understanding the functions of portal fibroblasts will require us to view them as more than just an alternative to hepatic stellate cells in fibrosis.
门脉纤维母细胞是门管区的固有纤维母细胞，位于胆管周围的间充质中。它们在肝脏稳态和损伤反应中的作用尚未明确，存在争议。尽管在胆管纤维化的发展过程中，门脉纤维母细胞几乎肯定会产生肌纤维母细胞，但最近的谱系追踪研究表明，与肝星状细胞相比，它们对纤维化的贡献是有限的。门脉纤维母细胞的其他功能包括参与胆管周围干细胞龛、调节胆管细胞增殖以及特定基质蛋白的沉积。门脉纤维母细胞合成弹性蛋白和微原纤维的其他成分；这些可能具有结构作用，在胆管压力增加的情况下为胆管和脉管系统提供稳定性，或者可以调节纤维生成转化生长因子 β 的生物利用度，以响应损伤。从全身成纤维细胞群体的角度来看待门脉纤维母细胞，并研究其在基质沉积和上皮调节方面的特定龛位作用，可能会为它们在正常和受损肝脏中的贡献提供新的见解。要了解门脉纤维母细胞的功能，我们需要将它们不仅仅视为纤维化中肝星状细胞的替代物。
