Concentration, biosynthesis and degradation of collagen in idiopathic pulmonary fibrosis.
Concentration, biosynthesis and degradation of collagen in idiopathic pulmonary fibrosis.
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DOI：
10.1136/thx.41.5.355
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发表时间：
1986-05
期刊：
影响因子：
10
通讯作者：
M. Selman;M. Montaño;C. Ramos;R. Chapela
中科院分区：
文献类型：
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作者：
M. Selman;M. Montaño;C. Ramos;R. Chapela
尽管在体外和体内进行了一些研究，但肺纤维化的发病机制尚不清楚，一些与胶原生物化学相关的发现也存在争议。研究了11例特发性肺纤维化患者和6例对照组的胶原代谢。胶原蛋白浓度增加（平均值327（SD 76），与对照值185（18）微克/毫克干重相比，p <0.001），生物合成的正常值（平均值2.2%（0.8%）v 2.08%（0.5%），并且胶原溶解活性显著降低（平均0.07（0.04）v0.23（0.04）微克胶原降解/mg孵育的胶原，p小于0.001）。这些结果表明，胶原酶分解的改变在这种疾病中间质纤维化的维持和进展中起着重要作用。
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.