ArticleImmunology and cell biology2015
The activin A antagonist follistatin inhibits cystic fibrosis-like lung inflammation and pathology.
Article in Immunology and cell biology, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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Who cites it
14 citing papers in PubMed, 32 citations in OpenAlex.
- Activin A Inhibitory Peptides Suppress Fibrotic Pathways by Targeting Epithelial-Mesenchymal Transition and Fibroblast-Myofibroblast Transformation in Idiopathic Pulmonary Fibrosis.International journal of molecular sciences · 2025Article
- Activin A plays an essential role in migration and proliferation of hepatic stellate cells via Smad3 and calcium signaling.Scientific reports · 2024Article
- The Reign of Follistatin in Tumors and Their Microenvironment: Implications for Drug Resistance.Biology · 2024Review
- In mouse chronic pancreatitis CD25Nature communications · 2022Article
- Potential novel biomarkers for chronic lung allograft dysfunction and azithromycin responsive allograft dysfunction.Scientific reports · 2021Article
- Activin A as a Novel Chemokine Induces Migration of L929 Fibroblasts by ERK Signaling in Microfluidic Devices.Frontiers in cell and developmental biology · 2021Article
- Antagonistic effects of activin A and TNF-α on the activation of L929 fibroblast cells via Smad3-independent signaling.Scientific reports · 2020Article
- Urinary Activin A is a novel biomarker reflecting renal inflammation and tubular damage in ANCA-associated vasculitis.PloS one · 2019Article
- Activin in acute pancreatitis: Potential risk-stratifying marker and novel therapeutic target.Scientific reports · 2017Article
- Activin Biology After Lung Transplantation.Transplantation direct · 2017Article
- Targeting TGF-β Mediated SMAD Signaling for the Prevention of Fibrosis.Frontiers in pharmacology · 2017Review
- Activins and Inhibins: Roles in Development, Physiology, and Disease.Cold Spring Harbor perspectives in biology · 2016Review
- Substantial Increases Occur in Serum Activins and Follistatin during Lung Transplantation.PloS one · 2016Article
- The immunoregulatory and fibrotic roles of activin A in allergic asthma.Clinical and experimental allergy : journal of the British Society for Allergy and Clinical Immunology · 2015Review
Corrections and comments
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Authors and funding
10 authors at 4 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Cystic fibrosis (CF) is the most common life-limiting genetically acquired respiratory disorder. Patients with CF have thick mucus obstructing the airways leading to recurrent infections, bronchiectasis and neutrophilic airway inflammation culminating in deteriorating lung function. Current management targets airway infection and mucus clearance, but despite recent advances in care, life expectancy is still only 40 years. We investigated whether activin A is elevated in CF lung disease and whether inhibiting activin A with its natural antagonist follistatin retards lung disease progression. We measured serum activin A levels, lung function and nutritional status in CF patients. We studied the effect of activin A on CF lung pathogenesis by treating newborn CF transgenic mice (β-ENaC) intranasally with the natural activin A antagonist follistatin. Activin A levels were elevated in the serum of adult CF patients, and correlated inversely with lung function and body mass index. Follistatin treatment of newborn β-ENaC mice, noted for respiratory pathology mimicking human CF, decreased the airway activin A levels and key features of CF lung disease including mucus hypersecretion, airway neutrophilia and levels of mediators that regulate inflammation and chemotaxis. Follistatin treatment also increased body weight and survival of β-ENaC mice, with no evidence of local or systemic toxicity. Our findings demonstrate that activin A levels are elevated in CF and provide proof-of-concept for the use of the activin A antagonist, follistatin, as a therapeutic in the long-term management of lung disease in CF patients.
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