Selman M, Montaño M, Ramos C, Chapela R
Thorax. 1986 May;41(5):355-9. doi: 10.1136/thx.41.5.355.
Despite several studies both in vitro and in vivo, the pathogenesis of pulmonary fibrosis is unclear and some findings related to the biochemistry of collagen are controversial. Collagen metabolism was studied in 11 patients with idiopathic pulmonary fibrosis and in six control subjects. There was an increase in collagen concentration (mean 327 (SD 76) compared with control values of 185 (18) micrograms/mg dry weight, p less than 0.001), normal values for biosynthesis (mean 2.2% (0.8%) v 2.08% (0.5%), and a noteworthy decrease in collagenolytic activity (mean 0.07 (0.04) v 0.23 (0.04) micrograms of collagen degraded per mg of collagen incubated, p less than 0.001). These results suggest that an alteration in enzymatic breakdown of collagen plays an important role in the maintenance and progression of interstitial fibrosis in this disease.
尽管在体外和体内都进行了多项研究，但肺纤维化的发病机制仍不清楚，一些与胶原蛋白生物化学相关的发现存在争议。对11例特发性肺纤维化患者和6例对照者的胶原蛋白代谢进行了研究。胶原蛋白浓度增加（平均327（标准差76），而对照值为185（18）微克/毫克干重，p<0.001），生物合成正常（平均2.2%（0.8%）对2.08%（0.5%）），且胶原蛋白分解活性显著降低（每毫克孵育的胶原蛋白降解的胶原蛋白平均量为0.07（0.04）对0.23（0.04）微克，p<0.001）。这些结果表明，胶原蛋白酶促分解的改变在该疾病间质纤维化的维持和进展中起重要作用。
