ArticlePediatric research2009
Murine model for cystic fibrosis bone disease demonstrates osteopenia and sex-related differences in bone formation.
Article in Pediatric research, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
What it found
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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.
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Who cites it
19 citing papers in PubMed, 53 citations in OpenAlex.
- The endocrine complications of cystic fibrosis.Nature reviews. Endocrinology · 2026Review
- Bone Disease in Cystic Fibrosis: Insights into Etiopathogenesis and Advances in Treatment Management.Journal of clinical medicine · 2025Review
- Patients With Cystic Fibrosis Undergoing Posterior Lumbar Fusion Are at an Increased Odds of Perioperative Complications.Journal of the American Academy of Orthopaedic Surgeons. Global research & reviews · 2025Article
- Cystic Fibrosis Bone Disease: The Interplay between CFTR Dysfunction and Chronic Inflammation.Biomolecules · 2023Review
- Endocrine Complications of Cystic Fibrosis.Clinics in chest medicine · 2022Review
- Animal models of cystic fibrosis in the era of highly effective modulator therapies.Current opinion in pharmacology · 2022Review
- The Effects of Ivacaftor on Bone Density and Microarchitecture in Children and Adults with Cystic Fibrosis.The Journal of clinical endocrinology and metabolism · 2021Observational
- Growth failure and treatment in cystic fibrosis.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2019Review
- Sex differences impact the lung-bone inflammatory response to repetitive inhalant lipopolysaccharide exposures in mice.Journal of immunotoxicology · 2018Article
- Children and adolescents with cystic fibrosis display moderate bone microarchitecture abnormalities: data from high-resolution peripheral quantitative computed tomography.Osteoporosis international : a journal established as result of cooperation between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA · 2017Article
- Reduced bone length, growth plate thickness, bone content, and IGF-I as a model for poor growth in the CFTR-deficient rat.PloS one · 2017Article
- Bone disease in cystic fibrosis: new pathogenic insights opening novel therapies.Osteoporosis international : a journal established as result of cooperation between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA · 2016Review
- Increased NF-κB Activity and Decreased Wnt/β-Catenin Signaling Mediate Reduced Osteoblast Differentiation and Function in ΔF508 Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Mice.The Journal of biological chemistry · 2015Article
- Cystic fibrosis-related bone disease: insights into a growing problem.Current opinion in endocrinology, diabetes, and obesity · 2013Review
- Article
- CFTR induces extracellular acid sensing in Xenopus oocytes which activates endogenous Ca²⁺-activated Cl⁻ conductance.Pflugers Archiv : European journal of physiology · 2011Article
- Cystic fibrosis growth retardation is not correlated with loss of Cftr in the intestinal epithelium.American journal of physiology. Gastrointestinal and liver physiology · 2011Article
- Pigs and humans with cystic fibrosis have reduced insulin-like growth factor 1 (IGF1) levels at birth.Proceedings of the National Academy of Sciences of the United States of America · 2010Article
- Strategies and First Advances in the Development of Prevascularized Bone Implants.Current molecular biology reportsReview
Corrections and comments
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Authors and funding
8 authors at 1 institution in 1 country.
Funding
Abstract
As the incidence of cystic fibrosis (CF) bone disease is increasing, we analyzed CF transmembrane conductance regulator (CFTR) deficient mice (CF mice) to gain pathogenic insights. In these studies comparing adult (14 wk) CF and C57BL/6J mice, both bone length and total area were decreased in CF mice. Metaphyseal trabecular and cortical density were also decreased, as well as diaphyseal cortical and total density. Trabecular bone volume was diminished in CF mice. Female CF mice revealed decreased trabecular width and number compared with C57BL/6J, whereas males demonstrated no difference in trabecular number. Female CF mice had reduced mineralizing surface and bone formation rates. Conversely, male CF mice had increased mineralizing surface, mineral apposition, and bone formation rates compared with C57BL/6J males. Bone formation rate was greater in males compared with female CF mice. Smaller bones with decreased density in CF, despite absent differences in osteoblast and osteoclast surfaces, suggest CF transmembrane conductance regulator influences bone cell activity rather than number. Differences in bone formation rate in CF mice are suggestive of inadequate bone formation in females but increased bone formation in males. This proanabolic observation in male CF mice is consistent with other clinical sex differences in CF.
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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.