ArticleAmerican journal of respiratory cell and molecular biology2016
Pathological Hyaluronan Matrices in Cystic Fibrosis Airways and Secretions.
Article in American journal of respiratory cell and molecular biology, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
22 citing papers in PubMed, 27 citations in OpenAlex.
- Inhaled high molecular weight hyaluronan ameliorates respiratory failure in acute COPD exacerbation: a pilot study.Respiratory research · 2021Trial
- Migration of immune cells in tumors and inflammation: molecular mechanisms and therapeutic targets.Signal transduction and targeted therapy · 2026Review
- Lymphatic disruption drives lung transplant fibrosis through interleukin-1-mediated hyaluronan accumulation.Science translational medicine · 2026Article
- A Narrative Review of Salivary Gland Extracellular Matrix and Sjögren's Syndrome: Research Status and Future Prospects.Biomolecules · 2026Review
- Hyaluronan: An Architect and Integrator for Cancer and Neural Diseases.International journal of molecular sciences · 2025Review
- Identification and validation of a threshold for early posttransplant bronchoalveolar fluid hyaluronan that distinguishes lung recipients at risk for chronic lung allograft dysfunction.The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation · 2025Article
- Role of Hyaluronic acid and its chemical derivatives in immunity during homeostasis, cancer and tissue regeneration.Seminars in immunopathology · 2024Review
- Human Mesenchymal Stem Cell (hMSC) Donor Potency Selection for the "First in Cystic Fibrosis" Phase I Clinical Trial (CEASE-CF).Pharmaceuticals (Basel, Switzerland) · 2023Article
- Dysfunctional Extracellular Matrix Remodeling Supports Perianal Fistulizing Crohn's Disease by a Mechanoregulated Activation of the Epithelial-to-Mesenchymal Transition.Cellular and molecular gastroenterology and hepatology · 2023Article
- Defining the versican interactome in lung health and disease.American journal of physiology. Cell physiology · 2022Review
- Biochemical, biophysical, and immunological characterization of respiratory secretions in severe SARS-CoV-2 infections.JCI insight · 2022Article
- Biochemical, Biophysical, and Immunological Characterization of Respiratory Secretions in Severe SARS-CoV-2 (COVID-19) Infections.medRxiv : the preprint server for health sciences · 2022Article
- Modulation of hyaluronan signaling as a therapeutic target in human disease.Pharmacology & therapeutics · 2022Review
- Enhancing Cystic Fibrosis Immune Regulation.Frontiers in pharmacology · 2021Article
- Article
- The Inter-α-Trypsin Inhibitor Family: Versatile Molecules in Biology and Pathology.The journal of histochemistry and cytochemistry : official journal of the Histochemistry Society · 2020Review
- Defective lung function following influenza virus is due to prolonged, reversible hyaluronan synthesis.Matrix biology : journal of the International Society for Matrix Biology · 2019Article
- Hyaluronan interactions with innate immunity in lung biology.Matrix biology : journal of the International Society for Matrix Biology · 2019Review
- Endothelial Progenitor Cells Modulate Inflammation-Associated Stroke Vasculome.Stem cell reviews and reports · 2019Article
- A Retrospective Analysis of the Cartilage Kunitz Protease Inhibitory Proteins Identifies These as Members of the Inter-α-Trypsin Inhibitor Superfamily with Potential Roles in the Protection of the Articulatory Surface.International journal of molecular sciences · 2019Article
Corrections and comments
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Authors and funding
7 authors at 6 institutions in 4 countries.
Funding
Abstract
Hyaluronan (HA) has been used in treatment of cystic fibrosis (CF) via a nebulizer and has demonstrated success in clinical outcomes. HA is an important glycosaminoglycan that is cross-linked by heavy chains (HCs) from inter-α-inhibitor during inflammation. HC cross-linked HA (HC-HA) becomes significantly more adhesive for leukocytes than non-cross-linked HA, which can enhance inflammation. Our studies tested the hypothesis that HC-HA is present in CF airways and that altered ratios of HC-HA to its degradation into relatively lower molecular weight HA contribute to the pathophysiology of chronic inflammation in CF. We evaluated the distribution, levels, and size of HC-HA within CF, healthy, and diseased control lung, bronchus, and sputum tissues by histological and biochemical approaches. HC-HA was significantly elevated in CF, with deposits around the pulmonary vasculature, airway submucosa, and in the stroma of the submucosal glands. The increased infiltration of leukocyte populations correlated with the distribution of HC-HA matrices in the airways. Elevated lung tissue HC-HA correlated with decreased HA levels in CF mucus and sputum compared with controls, suggesting that aberrant degradation and cross-linking of HA in lung tissue is a unique feature of CF. The accumulation and degradation of proinflammatory HC-HA in CF lung tissue suggests that aberrant HA catabolism and cross-linking may contribute to chronic inflammation in airway tissues and affect mucus viscosity in CF airways.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.