Role of lung pericytes and resident fibroblasts in the pathogenesis of pulmonary fibrosis.
Chi F. Hung,Geoffrey Linn,Yu Hua Chow,Akio Kobayashi,Kristen Mittelsteadt,William A. Altemeier,Sina A. Gharib,Lynn M. Schnapp,Jeremy S. Duffield +8 more
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TLDR
The lung contains an extensive population of Foxd1 progenitor-derived pericytes that are an important lung myofibroblast precursor population, and these studies suggest a distinct lineage of collagen-I(α)1-expressing resident fibroblasts that also expands after lung injury is a second major source of my ofibro Blasts.Abstract:
Rationale: The origin of cells that make pathologic fibrillar collagen matrix in lung disease has been controversial. Recent studies suggest mesenchymal cells may contribute directly to fibrosis.Objectives: To characterize discrete populations of mesenchymal cells in the normal mouse lung and to map their fate after bleomycin-induced lung injury.Methods: We mapped the fate of Foxd1-expressing embryonic progenitors and their progeny during lung development, adult homeostasis, and after fibrosing injury in Foxd1-Cre; Rs26-tdTomato-R mice. We studied collagen-I(α)1–producing cells in normal and diseased lungs using Coll-GFPTg mice.Measurements and Main Results: Foxd1-expressing embryonic progenitors enter lung buds before 13.5 days post-conception, expand, and form an extensive lineage of mesenchymal cells that have characteristics of pericytes. A collagen-I(α)1–expressing mesenchymal population of distinct lineage is also found in adult lung, with features of a resident fibroblast. In contrast to resident f...read more
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3e Journée française de la fibrose pulmonaire idiopathique. 19 septembre 2014
Vincent Cottin,Bruno Crestani,Claire Danel,Marie-Pierre Debray,Hilario Nunes,Venerino Poletti,G. Prévost,Jean-Michel Vergnon,Benoit Wallaert,J.F. Cordier +9 more
TL;DR: La question de la source de ces fibroblastes est régulièrement discutée depuis une quinzaine d’années and de two nouveaux acteurs ont été récemment identifiés.
Journal ArticleDOI
Wilms Tumor 1-Driven Fibroblast Activation and Subpleural Thickening in Idiopathic Pulmonary Fibrosis
Prathibha R. Gajjala,A. K. Singh,Varshini S Odayar,Harshavardhana H. Ediga,Francis X. McCormack,Satish K. Madala +5 more
TL;DR: In this paper , the key cellular and molecular events that contribute to (myo)fibroblast activation and subpleural thickening in IPF were discussed, highlighting the transcriptional programs involved in mesothelial to mesenchymal transformation and fibroblast dysfunction that can be targeted to alter the course of the progressive expansion of fibrotic lesions.
Journal ArticleDOI
p53 and Myofibroblast Apoptosis in Organ Fibrosis
TL;DR: In this article , the role of p53 dysregulation and apoptotic failure in myofibroblasts and demonstrate its consistent link to fibrotic disease development in all types of organ fibrosis.
Journal ArticleDOI
A roadmap for developing and engineering in vitro pulmonary fibrosis models
Mohammad Dabaghi,Mabel Barreiro Carpio,Neda Saraei,Jose M. Moran-Mirabal,Martin Kolb,Jeremy A. Hirota +5 more
TL;DR: In this article , the authors focus on the importance of extracellular matrix (ECM) components in the disease progression and highlight the state of the art in the field and future directions, which can be leveraged to understand better different mechanisms involved in IPF and develop drug candidates with improved efficacy.
Journal ArticleDOI
Mécanismes de la fibrose pulmonaire idiopathique
TL;DR: L’inflammation jouerait un rôle limité notamment dans les phases précoces of the maladie, mais pourrait contribuer à l’entretien du cercle vicieux de the fibrose.
References
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Pericytes: developmental, physiological, and pathological perspectives, problems, and promises.
TL;DR: The history of investigations into pericytes, the mural cells of blood microvessels, are reviewed, emerging concepts are indicated, and problems and promise are pointed out.
Journal ArticleDOI
Endothelial/Pericyte Interactions
TL;DR: This review focuses on the advancement in recent years of the understanding of intercellular communication between endothelial and mural cells with a focus on transforming growth factor α, angiopoietins, platelet-derived growth factor, spingosine-1-phosphate, and Notch ligands and their respective receptors.
Journal ArticleDOI
The role of pericytes in blood-vessel formation and maintenance
Gabriele Bergers,Steven Song +1 more
TL;DR: This review article describes the current knowledge about the nature of pericytes and their functions during vessel growth, vessel maintenance, and pathological angiogenesis.
Journal ArticleDOI
Fate Tracing Reveals the Pericyte and Not Epithelial Origin of Myofibroblasts in Kidney Fibrosis
Benjamin D. Humphreys,Benjamin D. Humphreys,Shuei-Liong Lin,Shuei-Liong Lin,Akio Kobayashi,Thomas E. Hudson,Brian T. Nowlin,Joseph V. Bonventre,Joseph V. Bonventre,M. Todd Valerius,Andrew P. McMahon,Jeremy S. Duffield,Jeremy S. Duffield +12 more
TL;DR: Data indicate that therapeutic strategies directly targeting pericyte differentiation in vivo may productively impact fibrotic kidney disease.
Journal ArticleDOI
Incidence and Prevalence of Idiopathic Pulmonary Fibrosis
TL;DR: The results suggest that idiopathic pulmonary fibrosis is probably more common in the United States than previously reported.
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