Shared and distinct mechanisms of fibrosis

JHW Distler, AH Györfi, M Ramanujam… - Nature Reviews …, 2019 - nature.com
Fibrosis is defined as an excessive deposition of connective tissue components and can
affect virtually every organ system, including the skin, lungs, liver and kidney. Fibrotic tissue …

Systemic sclerosis

Y Allanore, R Simms, O Distler… - Nature reviews Disease …, 2015 - nature.com
Systemic sclerosis is a complex autoimmune disease characterized by a chronic and
frequently progressive course and by extensive patient-to-patient variability. Like other …

Myofibroblast transcriptome indicates SFRP2hi fibroblast progenitors in systemic sclerosis skin

T Tabib, M Huang, N Morse, A Papazoglou… - Nature …, 2021 - nature.com
Skin and lung fibrosis in systemic sclerosis (SSc) is driven by myofibroblasts, alpha-smooth
muscle actin expressing cells. The number of myofibroblasts in SSc skin correlates with the …

Mesenchymal stem cells in fibrotic disease

E El Agha, R Kramann, RK Schneider, X Li, W Seeger… - Cell stem cell, 2017 - cell.com
Fibrosis is associated with organ failure and high mortality and is commonly characterized
by aberrant myofibroblast accumulation. Investigating the cellular origin of myofibroblasts in …

Signaling in fibrosis: TGF-β, WNT, and YAP/TAZ converge

B Piersma, RA Bank, M Boersema - Frontiers in medicine, 2015 - frontiersin.org
Chronic organ injury leads to fibrosis and eventually organ failure. Fibrosis is characterized
by excessive synthesis, remodeling, and contraction of extracellular matrix produced by …

TGF-β-induced endothelial-mesenchymal transition in fibrotic diseases

E Pardali, G Sanchez-Duffhues… - International journal of …, 2017 - mdpi.com
Fibrotic diseases are characterized by net accumulation of extracellular matrix proteins in
affected organs leading to their dysfunction and ultimate failure. Myofibroblasts have been …

Pathogenesis of systemic sclerosis: recent insights of molecular and cellular mechanisms and therapeutic opportunities

J Varga, M Trojanowska… - Journal of Scleroderma …, 2017 - journals.sagepub.com
Systemic sclerosis (SSc) is a complex disease characterized by early microvascular
abnormalities, immune dysregulation and chronic inflammation, and subsequent fibrosis of …

The epithelial-to-mesenchymal transition as a possible therapeutic target in fibrotic disorders

J Di Gregorio, I Robuffo, S Spalletta… - Frontiers in cell and …, 2020 - frontiersin.org
Fibrosis is a chronic and progressive disorder characterized by excessive deposition of
extracellular matrix, which leads to scarring and loss of function of the affected organ or …

Two-way conversion between lipogenic and myogenic fibroblastic phenotypes marks the progression and resolution of lung fibrosis

E El Agha, A Moiseenko, V Kheirollahi, S De Langhe… - Cell stem cell, 2017 - cell.com
Idiopathic pulmonary fibrosis (IPF) is a form of progressive interstitial lung disease with
unknown etiology. Due to a lack of effective treatment, IPF is associated with a high mortality …

Targeting TGF-β signaling for the treatment of fibrosis

AH Györfi, AE Matei, JHW Distler - Matrix biology, 2018 - Elsevier
Transforming growth factor-β (TGF-β) is widely recognized as a core pathway of fibrosis.
Inhibition of TGF-β signaling may thus offer potential for antifibrotic therapies. Long-term …