INSERM, Unité 1552, Paris, France.
Département Hospitalo-Universitaire Fibrosis, Inflammation, and Remodeling in Renal and Respiratory Diseases (FIRE), Paris, France.
FASEB J. 2018 Mar;32(3):1250-1264. doi: 10.1096/fj.201700583R. Epub 2018 Jan 3.
Idiopathic pulmonary fibrosis (IPF) is characterized by the deposition of excessive extracellular matrix and the destruction of lung parenchyma, resulting from an aberrant wound-healing response. Although IPF is often associated with an imbalance in protease activity, the mechanisms underlying the sustained repair mechanisms are not fully understood. Here, we addressed the role of the recently identified, membrane-anchored serine protease human airway trypsin-like protease (HAT). In the present study, we show that both HAT expression and activity were up-regulated in human IPF specimens. Next, adenoviral overexpression of HAT before bleomycin challenge attenuated lung injury as well as extracellular matrix deposition in the bleomycin-induced pulmonary fibrosis model. In vitro, HAT prevented specific fibrosis-associated responses in primary human pulmonary fibroblasts and induced the expression of mediators associated with the prostaglandin E2 pathway. Altogether, our findings suggested that HAT could have a protective role in IPF and other fibrotic lung disorders.-Menou, A., Flajolet, P., Duitmen, J., Justet, A., Moog, S., Jaillet, M., Tabèze, L., Solhonne, B., Garnier, M., Mal, H., Mordant, P., Castier, Y., Cazes, A., Sallenave, J.-M., Mailleux, A. A., Crestani, B. Human airway trypsin-like protease exerts potent, antifibrotic action in pulmonary fibrosis.
特发性肺纤维化 (IPF) 的特征是细胞外基质过度沉积和肺实质破坏,这是由于异常的伤口愈合反应所致。尽管 IPF 通常与蛋白酶活性失衡有关,但持续修复机制的机制尚不完全清楚。在这里,我们研究了最近发现的膜锚定丝氨酸蛋白酶人气道胰蛋白酶样蛋白酶 (HAT) 的作用。在本研究中,我们表明 HAT 的表达和活性在人特发性肺纤维化标本中均上调。接下来,在博来霉素攻击前过表达 HAT 可减轻博来霉素诱导的肺纤维化模型中的肺损伤和细胞外基质沉积。在体外,HAT 可防止原代人肺成纤维细胞中特定的纤维化相关反应,并诱导与前列腺素 E2 途径相关的介质的表达。总之,我们的研究结果表明,HAT 可能在特发性肺纤维化和其他纤维性肺疾病中具有保护作用。