Pulmonary fibrosis: something old, something new…still waiting for a breakthrough

Feghali‐Bostwick, Carol

doi:10.1152/ajplung.00366.2020

Cited by 4 publications

(3 citation statements)

References 22 publications

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“…The mechanisms underlying the pathogenesis of IPF are unclear (6)(7)(8). The main pathologic features include the collapse and obliteration of distal small airways, the proliferation and accumulation of fibroblast/myofibroblast cells, and increased collagen deposition in the alveolar spaces (9,10).…”

Section: Introductionmentioning

confidence: 99%

Targeting ATP12A, a non-gastric proton pump alpha subunit, for idiopathic pulmonary fibrosis treatment

Abdelgied

Uhl

Chen

et al. 2022

Preprint

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Idiopathic Pulmonary Fibrosis (IPF) is a pathological condition of unknown etiology which results from injury to the lung and an ensuing fibrotic response that leads to thickening of the alveolar walls and obliteration of the alveolar space. The pathogenesis is not clear and there are currently no effective therapies for IPF. Small airway disease and mucus accumulation are prominent features in IPF lungs, similar to Cystic Fibrosis (CF) lung disease. The ATP12A gene encodes the alpha-subunit of the non-gastric H+, K+-ATPase, which functions to acidify the airway surface fluid and impairs mucociliary transport function in cystic fibrosis patients. We hypothesize that the ATP12A protein may play a role in the pathogenesis of IPF. Our studies demonstrate that ATP12A protein is overexpressed in distal small airways from IPF patient lungs compared to normal human lungs. In addition, overexpression of the ATP12A protein in mouse lungs worsened the bleomycin (BLEO)-induced experimental pulmonary fibrosis. This was prevented by a potassium-competitive proton pump blocker, vonoprazan (VON). This data supports the concept that the ATP12A protein plays an important role in the pathogenesis of lung fibrosis. Inhibition of the ATP12A protein has the potential as a novel therapeutic strategy in IPF.

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Section: Introductionmentioning

confidence: 99%

Targeting ATP12A, a non-gastric proton pump alpha subunit, for idiopathic pulmonary fibrosis treatment

Abdelgied

Uhl

Chen

et al. 2022

Preprint

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“…Bruce McManus, MD 1,2 and Darryl Knight, PhD 3,4,5, 6 1 Prevention of Organ Failure (PROOF) Centre of Excellence, Vancouver, BC, Canada; 2 Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, BC, Canada; 3 Providence Health Care Research Institute, Vancouver, BC, Canada;…”

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confidence: 99%

“…A key factor underlying the barren therapeutic landscape is that the cause(s) of the disease remain unknown. Several lung cell types including alveolar epithelial cells, fibroblasts, macrophages and endothelial cells have been implicated in the development and progression of fibrosis, and changes in cellular metabolism leading to altered mitochondrial oxidation and dysregulated protein folding have been increasingly implicated in remodelling of lung architecture and fibrosis 2 . Yet, the mechanisms of disease initiation and progression remain elusive.…”

mentioning

confidence: 99%

Long‐chain fatty acids are bad in IPF, or are they?

McManus

Knight

2020

Respirology

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World health observances in September 2020: sepsis, the lung and heart, and pulmonary fibrosis in focus

Nardiello

Morty

2020

American Journal of Physiology-Lung Cellular and Molecular Phys

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World Lung Day was first conceived at the 2016 Assembly Meeting of the Forum of International Respiratory Societies (FIRS) held in Kyoto, Japan. FIRS had previously published a report (11) highlighting the global burden of respiratory disease, and on the basis of that report, World Lung Day was intended to act as a global Call to Action for policy makers, health care stakeholders, and civil society. The first World Lung Day was held on

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Pulmonary fibrosis: something old, something new…still waiting for a breakthrough

Cited by 4 publications

References 22 publications

Targeting ATP12A, a non-gastric proton pump alpha subunit, for idiopathic pulmonary fibrosis treatment

Targeting ATP12A, a non-gastric proton pump alpha subunit, for idiopathic pulmonary fibrosis treatment

Long‐chain fatty acids are bad in IPF, or are they?

World health observances in September 2020: sepsis, the lung and heart, and pulmonary fibrosis in focus

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