ArticleAmerican journal of physiology. Gastrointestinal and liver physiology2011
Cystic fibrosis growth retardation is not correlated with loss of Cftr in the intestinal epithelium.
Article in American journal of physiology. Gastrointestinal and liver physiology, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 33 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.
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Who cites it
33 citing papers in PubMed, 45 citations in OpenAlex.
- Impact of Genetics on Endocrine Manifestations of Cystic Fibrosis.Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists · 2026Review
- Diagnosis and Emerging Biomarkers of Cystic Fibrosis-Related Kidney Disease (CFKD).Journal of clinical medicine · 2025Review
- A W1282X cystic fibrosis mouse allows the study of pharmacological and gene-editing therapeutics to restore CFTR function.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2025Article
- Think Beyond the Room: Measuring Relative Humidity in the Home Cage and Its Impact on Reproduction in Laboratory Mice,Animals : an open access journal from MDPI · 2024Article
- Impaired intestinal free fatty acid transport followed by chylomicron malformation, not pancreatic insufficiency, cause metabolic defects in cystic fibrosis.Journal of lipid research · 2024Article
- Fecal dysbiosis and inflammation in intestinal-specific Cftr knockout mice on regimens preventing intestinal obstruction.Physiological genomics · 2024Article
- CFTR dysfunction in smooth muscle drives TGFβ dependent airway hyperreactivity.Respiratory research · 2023Article
- Animal models of cystic fibrosis in the era of highly effective modulator therapies.Current opinion in pharmacology · 2022Review
- Enhancing Cystic Fibrosis Immune Regulation.Frontiers in pharmacology · 2021Article
- CFTR dysregulation drives active selection of the gut microbiome.PLoS pathogens · 2020Article
- A BAC Transgene Expressing Human CFTR under Control of Its Regulatory Elements Rescues Cftr Knockout Mice.Scientific reports · 2019Article
- Alterations of skeletal muscle bioenergetics in a mouse with F508del mutation leading to a cystic fibrosis-like condition.American journal of physiology. Endocrinology and metabolism · 2019Article
- Electrochemical measurement of membrane cholesterol correlates with CFTR function and is HDAC6-dependent.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2019Article
- Genetic association and transcriptome integration identify contributing genes and tissues at cystic fibrosis modifier loci.PLoS genetics · 2019Article
- Small adipose stores in cystic fibrosis mice are characterized by reduced cell volume, not cell number.American journal of physiology. Gastrointestinal and liver physiology · 2018Article
- Kcnn4 is a modifier gene of intestinal cystic fibrosis preventing lethality in the Cftr-F508del mouse.Scientific reports · 2018Article
- A G542X cystic fibrosis mouse model for examining nonsense mutation directed therapies.PloS one · 2018Article
- Improved Growth Patterns in Cystic Fibrosis Mice after Loss of Histone Deacetylase 6.Scientific reports · 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
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Authors and funding
5 authors at 2 institutions in 2 countries.
Funding
Abstract
Maldigestion due to exocrine pancreatic insufficiency leads to intestinal malabsorption and consequent malnutrition, a mechanism proposed to cause growth retardation associated with cystic fibrosis (CF). However, although enzyme replacement therapy combined with increased caloric intake improves weight gain, the effect on stature is not significant, suggesting that growth retardation has a more complex etiology. Mouse models of CF support this, since these animals do not experience exocrine pancreatic insufficiency yet are growth impaired. Cftr absence from the intestinal epithelium has been suggested as a primary source of growth retardation in CF mice, a concept we directly tested by generating mouse models with Cftr selectively inactivated or restored in intestinal epithelium. The relationship between growth and functional characteristics of the intestines, including transepithelial electrophysiology, incidence of intestinal obstruction, and histopathology, were assessed. Absence of Cftr exclusively from intestinal epithelium resulted in loss of cAMP-stimulated short-circuit current, goblet cell hyperplasia, and occurrence of intestinal obstructions but only slight and transient impaired growth. In contrast, specifically restoring Cftr to the intestinal epithelium resulted in restoration of ion transport and completely protected against obstruction and histopathological anomalies, but growth was indistinguishable from CF mice. These results indicate that absence of Cftr in the intestinal epithelium is an important contributor to the intestinal obstruction phenotype in CF but does not correlate with the observed growth reduction 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.