ReviewThe international journal of biochemistry & cell biology2014
Cystic fibrosis: an inherited disease affecting mucin-producing organs.
Review in The international journal of biochemistry & cell biology, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 58 papers, 1 of them a synthesis that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
58 citing papers in PubMed, 1 synthesis or guideline pooled it, 107 citations in OpenAlex.
- Pharmacokinetics in Patients with Cystic Fibrosis: A Systematic Review of Data Published Between 1999 and 2019.Clinical pharmacokinetics · 2020Pooled it
- Mitochondria Meet the Lung Microbiome: A Bidirectional Dialogue in Inflammation and Respiratory Diseases.Biomedicines · 2026Review
- Cystic fibrosis and inflammatory bowel disease: parallels in gut physiology and microbiota.Journal of bacteriology · 2025Review
- The Role of Mucins in Cancer and Cancer Progression: A Comprehensive Review.Current issues in molecular biology · 2025Review
- Breaking Down the Bottlebrush: Atomically Detailed Structural Dynamics of Mucins.Journal of chemical information and modeling · 2024Article
- The Tmem16a chloride channel is required for mucin maturation after secretion from goblet-like cells in the Xenopus tropicalis tadpole skin.Scientific reports · 2024Article
- Review
- TRPV4 Channel Modulators as Potential Drug Candidates for Cystic Fibrosis.International journal of molecular sciences · 2024Review
- Vitamin D for glycemic control following an acute pulmonary exacerbation: A secondary analysis of a multicenter, double-blind, randomized, placebo-controlled trial in adults with cystic fibrosis.medRxiv : the preprint server for health sciences · 2024Article
- Fecal dysbiosis and inflammation in intestinal-specific Cftr knockout mice on regimens preventing intestinal obstruction.Physiological genomics · 2024Article
- Development of the Pancreatic Ducts and Their Contribution to Organogenesis.Advances in anatomy, embryology, and cell biology · 2024Review
- Measuring Biophysical Properties of Cilia Motility from Mammalian Tissues via Quantitative Video Analysis Methods.Methods in molecular biology (Clifton, N.J.) · 2024Article
- Pharmacodynamic Model of the Dynamic Response ofBiomedicines · 2023Article
- High-throughput quantification of microbial-derived organic acids in mucin-rich samples via reverse phase high performance liquid chromatography.Journal of medical microbiology · 2023Article
- Mucus aberrant properties in CF: Insights from cells and animal models.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2023Review
- PLGA-Based Micro/Nanoparticles: An Overview of Their Applications in Respiratory Diseases.International journal of molecular sciences · 2023Review
- CDK8 and CDK19 act redundantly to control the CFTR pathway in the intestinal epithelium.EMBO reports · 2023Article
- High-throughput functional assay in cystic fibrosis patient-derived organoids allows drug repurposing.ERJ open research · 2023Article
- Mucins and CFTR: Their Close Relationship.International journal of molecular sciences · 2022Review
- Mucus-targeting therapies of defective mucus clearance for cystic fibrosis: A short review.Current opinion in pharmacology · 2022Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
3 authors at 3 institutions in 2 countries.
Funding
Abstract
Our current understanding of cystic fibrosis (CF) has revealed that the biophysical properties of mucus play a considerable role in the pathogenesis of the disease in view of the fact that most mucus-producing organs are affected in CF patients. In this review, we discuss the potential causal relationship between altered cystic fibrosis transmembrane conductance regulator (CFTR) function and the production of mucus with abnormal biophysical properties in the intestine and lungs, highlighting what has been learned from cell cultures and animal models that mimic CF pathogenesis. A similar cascade of events, including mucus obstruction, infection and inflammation, is common to all epithelia affected by impaired surface hydration. Hence, the main structural components of mucus, namely the polymeric, gel-forming mucins, are critical to the onset of the disease. Defective CFTR leads to epithelial surface dehydration, altered pH/electrolyte composition and mucin concentration. Further, it can influence mucin transition from the intracellular to extracellular environment, potentially resulting in aberrant mucus gel formation. While defective HCO3(-) production has long been identified as a feature of CF, it has only recently been considered as a key player in the transition phase of mucins. We conclude by examining the influence of mucins on the biophysical properties of CF sputum and discuss existing and novel therapies aimed at removing mucus from the lungs. This article is part of a Directed Issue entitled: Cystic Fibrosis: From o-mics to cell biology, physiology, and therapeutic advances.
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What OpenQuestion holds
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.