In:
eLife, eLife Sciences Publications, Ltd, Vol. 7 ( 2018-07-03)
Abstract:
Idiopathic pulmonary fibrosis (IPF) is a devastating disease of the lung, which scars the tissue and gradually destroys the organ, ultimately leading to death. It is still unclear what exactly causes this scarring, but it is thought that increasing amounts of proteins in the space surrounding the cells of the lungs, the extracellular matrix, could play a role. These proteins, including collagen, normally form a ‘scaffold’ to stabilize cells, but if they accumulate uncontrollably, they can render tissues rigid. It has been assumed that these changes are a consequence of the disease. However, recent evidence suggests that the increased stiffness itself could stimulate cells to produce even more extracellular matrix, driving the progression of the disease. A better understanding of what exactly causes the tissue to become gradually stiffer may identify new ways to block the progression of IPF. Now, Jones et al. compared measurements of the tissue stiffness and the collagen structure taken from samples of patients with IPF. The results showed that the collagen fibres were faulty and had an abnormal shape. This suggests that these problems, rather than an increased amount of collagen, alter the flexibility of the lung tissue. Jones et al. also found that a specific family of proteins, which helps to connect the collagen fibres, was increased in the tissue of patients with IPF. When these proteins were blocked with a newly developed drug, the collagen structure returned to normal and the stiffness of the tissue decreased. As a consequence, the lung capacity improved. This suggests that treatment approaches that help to maintain a normal collagen structure, may in future prevent the stiffening of the lung tissue and so limit feed-forward mechanisms that drive progressive IPF. Moreover, it indicates that measurements of the structure of collagen rather than the its total concentration could serve as a more suitable indicator for the disease.
Type of Medium:
Online Resource
ISSN:
2050-084X
DOI:
10.7554/eLife.36354.001
DOI:
10.7554/eLife.36354.002
DOI:
10.7554/eLife.36354.003
DOI:
10.7554/eLife.36354.004
DOI:
10.7554/eLife.36354.005
DOI:
10.7554/eLife.36354.006
DOI:
10.7554/eLife.36354.007
DOI:
10.7554/eLife.36354.008
DOI:
10.7554/eLife.36354.009
DOI:
10.7554/eLife.36354.010
DOI:
10.7554/eLife.36354.011
DOI:
10.7554/eLife.36354.012
DOI:
10.7554/eLife.36354.013
DOI:
10.7554/eLife.36354.014
DOI:
10.7554/eLife.36354.015
Language:
English
Publisher:
eLife Sciences Publications, Ltd
Publication Date:
2018
detail.hit.zdb_id:
2687154-3
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