Biochemical pathways leading to collagen deposition in pulmonary fibrosis.
Fibrosis in the lung is well described histologically. There is destruction of the normal architecture with the appearance of inflammatory cells and connective tissue components, particularly collagen. Biochemical evidence for an increased deposition of collagen in man has been demonstrated in patients with both acute and chronic forms of pulmonary fibrosis. Studies of collagen metabolism in man are equivocal but there is convincing evidence for an increased synthesis rate in animal models of pulmonary fibrosis. Collagen degradation has been little studied but may be important, given the recent evidence indicating quite rapid turnover of lung collagen and a decreased degradation of collagen in experimental disease. The distribution of collagen types has been studied in man, where there is some evidence for the production of type III collagen in the early active phase of disease with a preponderance of type I collagen in the late stages. The cellular mechanisms leading to these changes are uncertain but the alveolar macrophage may play a central role, since it is capable of releasing factors which expand the fibroblast population as well as attracting new fibroblasts to the site of injury. These pathways are described for what is essentially the normal physiological response of scar formation, which has pathological consequences in the lung, a tissue requiring thin membranes at its epithelial and endothelial surfaces, in order to perform its main function of gas exchange.
D002450:Cell Communication; D003094:Collagen / Q000302:isolation & purification / Q000378:metabolism*; D006801:Humans; D007249:Inflammation; D008264:Macrophages / Q000502:physiology; D011658:Pulmonary Fibrosis / Q000378:metabolism* / Q000503:physiopathology / Q000628:therapy
D003094:Collagen