Exact(10)
However, due to the complexity and uncertainties intrinsic to IPF, it is essential that each therapeutic strategy be tailored to the individual patient, after evaluation of potential benefits and risks.
62, 63 Interestingly, while the MUC5B promoter polymorphism appears to confer increased risk of developing IPF, it may be associated with improved survival in established IPF.
Foremost, in attempting to replicate a complex and poorly understood disease such as IPF, it is nearly impossible to fully recapitulate the exact the nature of the disease.
Although there are no well-established categories of disease severity in IPF, it may be hypothesized that patients receiving supplemental oxygen represent patients with more severe disease.
Even if the BLM-induced pulmonary fibrosis does not represent a strictly equivalent of IPF, it constitutes a polyvalent model that produces morphological alterations of lung fibrosis with a robust reproducibility [ 12].
In cases of familial IPF, it was shown that mutations of the surfactant protein-C gene (SFTRC) lead to accumulation of misfolded proteins, induction of ER stress, and apoptosis of AEC II [ 50– 50].
Similar(50)
While mechanical features of the antenna may have evolved to optimally detect conspecific IPFs, it has been hypothesized that the brain is responsible for recognizing conspecific IPIs (Riabinina et al., 2011).
Desquamative interstitial pneumonitis (DIP) may rarely mimic IPF, as it may present with subpleural basal minor reticulation.
TGF-β expression is upregulated at the sites of the epithelial injury in IPF, thus it is likely a factor involved in EMT.
This suggests that although purinergic remodeling exists in both COPD and IPF patients, it is more likely that there is a causative role for A2BR driven mediator expression in IPF than in COPD.
Although there is compelling evidence that ER stress plays a role in IPF pathogenesis, it is also clear that other factors must be involved.
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