Pulmonary fibrosis: patterns and perpetrators

Noble, Paul W.; Barkauskas, Christina E.; Jiang, Dianhua

doi:10.1172/jci60323

Cited by 482 publications

(416 citation statements)

References 99 publications

Supporting

Mentioning

386

Contrasting

Unclassified

Order By: Relevance

“…2,3,6,9,10,12e16 Such broad adaptability is proposed to enable pericytes and resident fibroblasts to repeatedly adjust to support growing, injured, or regenerating vascular or airway structures. 2,6,13 Figure 1 Tissue architecture and mesenchymal lineages in the lung. A: Electron microscopy photomicrographs of normal mouse lung showing fibroblast processes abutting epithelium and pericyte processes attached to endothelium and within a loose capillary basement membrane.…”

Section: Characteristics Of Lung Pericytes and Resident Fibroblastsmentioning

confidence: 99%

“…2,13 Although these types of cells can assume a myofibroblast identity, key lineage-tracing experiments demonstrate that the pericytes and resident fibroblasts are the predominant source of myofibroblasts in the lung during development, in its mature healthy state, and after injury. 2,9,10,13 Pericytes and resident lung fibroblasts can be identified independently of their location, with appropriate criteria for excluding other cell types, by their expression of markers, including PDGF receptor (PDGFR) b and a, CD146, NG2, a-SMA, collagen I(a)1 (Col1), vimentin, and desmin (Table 1). 2,3,9,10,17 However, the fidelity of these markers varies by context.…”

Section: Characteristics Of Lung Pericytes and Resident Fibroblastsmentioning

confidence: 99%

“…15,53,64 Fibrotic lung diseases are a growing and serious cause of morbidity and mortality with scant therapeutic options. 13,78,79 Pollutants, such as tobacco smoke or asbestos, autoimmune diseases, and inflammatory disorders, directly cause lung fibrosis. When injured, lung epithelial cells release a diverse and potent assortment of factors that can activate fibroblasts, including TGF-b, connective tissue growth factor, prostaglandin E 2 (PGE 2 ), sonic hedgehog (SHH), WNT1 inducible signaling pathway protein 1 (WISP1), and IL-1a.…”

Section: Myofibroblasts In Lung Fibrosis and Degenerationmentioning

confidence: 99%

“…82 A strengthening hypothesis to generally explain fibrotic lung diseases is that injury or infection overcomes an already stressed epithelium's capacity for recuperation and engages innate immune pathways that may circumstantially promote or protect from harm. 13,15,53,64,75,78,81 Myofibroblasts are the key effector cell type in fibrotic disease because they are dominant builders and remodelers of extracellular matrix that also exert strong force on their surroundings. 9,10,12e14,27,30,38,53,64,78,83 Myofibroblasts may be distinguished by expressing a-SMA and similar proteins in common with muscle, forming stress fibers, and by contractility.…”

Section: Myofibroblasts In Lung Fibrosis and Degenerationmentioning

confidence: 99%

See 3 more Smart Citations

Lung Pericytes and Resident Fibroblasts

Barron

Gharib

Duffield

2016

The American Journal of Pathology

109

View full text Add to dashboard Cite

Pericytes, resident fibroblasts, and mesenchymal stem cells are poorly described cell populations. They have recently been characterized in much greater detail in rodent lungs and have been shown to play important roles in development, homeostasis, response to injury and pathogens, as well as recovery from damage. These closely related mesenchymal cell populations form extensive connections to the lung's internal structure, as well as its internal and external surfaces. They generate and remodel extracellular matrix, coregulate the vasculature, help maintain and restore the epithelium, and act as sentries for the immune system. In this review, we revisit these functions in light of significant advances in characterizing and tracking lung fibroblast populations in rodents. Lineage tracing experiments have mapped the heritage, identified functions that discriminate lung pericytes from resident fibroblasts, identified a subset of mesenchymal stem cells, and shown these populations to be the predominant progenitors of pathological fibroblasts and myofibroblasts in lung diseases. These findings point to the importance of resident lung mesenchymal populations as therapeutic targets in acute lung injury as well as fibrotic and degenerative diseases. Far from being passive and quiescent, pericytes and resident fibroblasts are busily sensing and responding, through diverse mechanisms, to changes in lung health and function. (Am J Pathol 2016 http://dx

show abstract

Section: Characteristics Of Lung Pericytes and Resident Fibroblastsmentioning

confidence: 99%

Section: Characteristics Of Lung Pericytes and Resident Fibroblastsmentioning

confidence: 99%

Section: Myofibroblasts In Lung Fibrosis and Degenerationmentioning

confidence: 99%

Section: Myofibroblasts In Lung Fibrosis and Degenerationmentioning

confidence: 99%

See 2 more Smart Citations

Lung Pericytes and Resident Fibroblasts

Barron

Gharib

Duffield

2016

The American Journal of Pathology

109

View full text Add to dashboard Cite

show abstract

“…By contrast, whilst ILC2 appear to have a benign and restorative effect following viral infection in the lung, they have also been implicated in the pathogenesis of lung fibrosis in animal models and are present in the lungs of patients suffering from idiopathic pulmonary fibrosis (Hams et al 2014), a condition believed to be driven by deregulated repair mechanisms (Noble et al 2012). Similarly, ILC2 have been implicated in the pathogenesis of hepatic fibrosis.…”

Section: Ilc2 Responses In Infectionmentioning

confidence: 99%

Innate Lymphoid Cells in Type 2 Immune Responses

Mirchandani

Salmond

2014

Arch. Immunol. Ther. Exp.

View full text Add to dashboard Cite

In recent years, several distinct innate lymphoid cell populations (ILC) have been characterized in mice and humans. Group 2 ILC function as a rapid responder population in type 2 immune responses. Thus, a wealth of data has implicated an important role for ILC2 in immunity to parasitic infection and in immune pathology in inflammatory and allergic responses. In this review, we describe recent progress in our understanding of the development and ontogeny of ILC2 populations and the mechanisms by which these cells function in a variety of infection and disease settings. Finally, we emphasize recent findings indicating functional interactions between these innate cells and their adaptive CD4 ? Th2 cell counterparts.

show abstract

Fibroblast growth factor 2 acts as an upstream regulator of inhibition of pulmonary fibroblast activation

et al. 2023

View full text Add to dashboard Cite

Fibroblast growth factor (FGF) signaling plays a crucial role in lung development and repair. FGF2 can inhibit fibrotic gene expression and suppress the differentiation of pulmonary fibroblasts (PFs) into myofibroblasts in vitro, suggesting that FGF2 is a potential target for inhibiting pulmonary fibrosis. To gain deeper insights into the molecular mechanism underlying FGF2‐mediated regulation of PFs, we performed mRNA sequencing analysis to systematically and globally uncover the regulated genes and biological functions of FGF2 in PFs. Gene ontology analysis revealed that the differentially expressed genes regulated by FGF2 were enriched in multiple cellular functions including extracellular matrix (ECM) organization, cytoskeleton formation, β‐catenin‐independent Wnt signaling pathway, supramolecular fiber organization, epithelial cell proliferation and cell adhesion. Gene set enrichment analysis (GSEA) and cellular experiments confirmed that FGF2 can suppress ECM and actin filament organization and increase PFs proliferation. Taken together, these findings indicate that FGF2 acts as an upstream regulator of the inhibition of PFs activation and may play a regulatory role in pulmonary fibrosis.

show abstract

Pulmonary fibrosis: patterns and perpetrators

Cited by 482 publications

References 99 publications

Lung Pericytes and Resident Fibroblasts

Lung Pericytes and Resident Fibroblasts

Innate Lymphoid Cells in Type 2 Immune Responses

Fibroblast growth factor 2 acts as an upstream regulator of inhibition of pulmonary fibroblast activation

Contact Info

Product

Resources

About