Extracellular matrix in normal and fibrotic human lungs.
Extracellular matrix in normal and fibrotic human lungs.
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正常和纤维化人肺中的细胞外基质。
DOI：
10.1164/arrd.1985.131.2.281
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发表时间：
1985
期刊：
影响因子：
--
通讯作者：
Striker,GE
中科院分区：
文献类型：
--
作者：
Raghu,G;Striker,LJ;Hudson,LD;Striker,GE
用亲和纯化的抗人I型、III型、IV型胶原多克隆抗体和层粘连蛋白，用间接免疫荧光法比较了10例正常肺和32例异常肺的细胞外基质(ECM)。在正常肺中，IV型胶原和层粘连蛋白沿上皮基底膜和内皮基底膜呈均匀的线状分布。肺泡间隔和间质中的III型胶原呈条带状分布，聚集在肺泡的入口环处。I型胶原在肺泡壁内呈不规则分布，不明显于III型胶原。在不以间质纤维化为特征的肺部疾病患者(n=15)中，所研究的ECM成分的分布基本上是正常的。在以间质纤维化为特征的肺部疾病中，如特发性肺纤维化(IPF)和成人呼吸窘迫综合征(ARDS)(n=17)，I型和III型胶原在扩张的间质中积聚。III型胶原最初在增厚的肺泡间隔和间质中占优势，而I型胶原在病程后期似乎是主要的胶原。病程早期基底膜被破坏，肺泡腔被类似于邻近间质的间质胶原物侵袭。
Polyclonal affinity-purified antibodies to human collagen types I, III, and IV, and laminin were used to compare the extracellular matrix (ECM) in 10 normal and 32 abnormal lungs by indirect immunofluorescence. In normal lungs, type IV collagen and laminin codistributed in a uniform linear pattern along the epithelial and endothelial basement membranes. Type III collagen was found within the alveolar septa and interstitium in an interrupted ribbonlike pattern and was aggregated at the entrance rings of the alveoli. Type I collagen was distributed irregularly within the alveolar wall and was less prominent than type III collagen. In patients with pulmonary disease not characterized by interstitial fibrosis (n = 15), the distribution of ECM components studied was essentially normal. In pulmonary disease in which interstitial fibrosis was the characteristic feature, such as idiopathic pulmonary fibrosis (IPF) and adult respiratory distress syndrome (ARDS) (n = 17), collagen types I and III accumulated in the expanded interstitium. Type III collagen was initially predominant in the thickened alveolar septa and interstitium, whereas type I collagen appeared to be the principal collagen at later stages in the disease course. The basement membrane was disrupted early in the disease course with invasion of the alveolar spaces by interstitial collagens similar in type to those present in the adjacent interstitium.