Biswas Rupa, Bunderson-Schelvan Melisa, Holian Andrij
Center for Environmental Health Sciences, The University of Montana, Skaggs Building 274, Missoula, MT 59812, USA.
Pulm Med. 2011;2011:105707. doi: 10.1155/2011/105707. Epub 2011 Jun 2.
Pulmonary fibrosis is a progressive, disabling disease with mortality rates that appear to be increasing in the western population, including the USA. There are over 140 known causes of pulmonary fibrosis as well as many unknown causes. Treatment options for this disease are limited due to poor understanding of the molecular mechanisms of the disease progression. However, recent progress in inflammasome research has greatly contributed to our understanding of its role in inflammation and fibrosis development. The inflammasome is a multiprotein complex that is an important component of both the innate and adaptive immune systems. Activation of proinflammatory cytokines following inflammasome assembly, such as IL-1β and IL-18, has been associated with development of PF. In addition, components of the inflammasome complex itself, such as the adaptor protein ASC have been associated with PF development. Recent evidence suggesting that the fibrotic process can be reversed via blockade of pathways associated with inflammasome activity may provide hope for future drug strategies. In this paper we will give an introduction to pulmonary fibrosis and its known causes. In addition, we will discuss the importance of the inflammasome in the development of pulmonary fibrosis as well as discuss potential future treatment options.
肺纤维化是一种进行性、致残性疾病,在包括美国在内的西方人群中,其死亡率似乎在上升。已知导致肺纤维化的病因超过140种,还有许多未知病因。由于对该疾病进展的分子机制了解不足,这种疾病的治疗选择有限。然而,近年来炎性小体研究的进展极大地促进了我们对其在炎症和纤维化发展中作用的理解。炎性小体是一种多蛋白复合体,是固有免疫系统和适应性免疫系统的重要组成部分。炎性小体组装后促炎细胞因子的激活,如白细胞介素-1β和白细胞介素-18,与肺纤维化的发展有关。此外,炎性小体复合体本身的成分,如衔接蛋白ASC,也与肺纤维化的发展有关。最近有证据表明,通过阻断与炎性小体活性相关的途径,纤维化过程可以逆转,这可能为未来的药物策略带来希望。在本文中,我们将介绍肺纤维化及其已知病因。此外,我们将讨论炎性小体在肺纤维化发展中的重要性,并探讨未来潜在的治疗选择。