Spitalieri P, Quitadamo M C, Orlandi A, Puxeddu E, Curradi G, Sangiuolo F
Department of Biomedicine and Prevention, Tor Vergata University of Rome, Rome, Italy.
Sarcoidosis Vasc Diffuse Lung Dis. 2013 Nov 22;30(3):177-85.
Idiopathic pulmonary fibrosis (IPF) is characterized by alveolar epithelial cell injury, type II cell activation, apoptosis and bronchiolar epithelial cell proliferation, accumulation of extracellular matrix and fibroblasts. No current animal model recapitulates all of these cardinal manifestation of the human disease. However, bleomycin instillation in mice lung by intranasal way (ITN) represents the best experimental model of pulmonary fibrosis in which alveolar pneumocytes type II (ATII) are usually depleted. The aim of this study was to test the possibility to recover acute lung fibrosis after transplantation of human embryonic type II derived-pneumocytes in a murine model of bleomycin-induced damage. Our results indicate the striking "clinical" beneficial effect of differentiated HUES-3 cells into ATII in terms of lung function, weight loss and mortality in injured mice, suggesting this stem cell therapy as a promising, systemic and specific treatment of human pulmonary fibrosis.
特发性肺纤维化（IPF）的特征是肺泡上皮细胞损伤、II型细胞活化、凋亡以及细支气管上皮细胞增殖、细胞外基质和成纤维细胞积聚。目前尚无动物模型能重现人类疾病的所有这些主要表现。然而，通过鼻内途径（ITN）向小鼠肺内滴注博来霉素是肺纤维化的最佳实验模型，其中II型肺泡上皮细胞（ATII）通常会减少。本研究的目的是测试在博来霉素诱导损伤的小鼠模型中，移植人胚胎来源的II型肺细胞后恢复急性肺纤维化的可能性。我们的结果表明，分化为ATII的HUES-3细胞在受损小鼠肺功能、体重减轻和死亡率方面具有显著的“临床”有益效果，提示这种干细胞疗法有望成为治疗人类肺纤维化的一种有前景的全身性特异性治疗方法。
