Evidence map›Paper›PMID 21857224›Full record

ReviewCurrent opinion in pulmonary medicine2011

New animal models of cystic fibrosis: what are they teaching us?

Nicholas W Keiser, John F Engelhardt

Abstract readReview
In one paragraph

Review in Current opinion in pulmonary medicine, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 71 papers.

0numbers the graph read from it
0cells of the map it votes in
71citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from 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.

2 · The registry

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.

3 · Its place in the literature

Who cites it

71 citing papers in PubMed.

  1. Pulmonary Vascular Dysfunctions in Cystic Fibrosis.Physiology (Bethesda, Md.) · 2024
    Review
  2. Pkhd1Journal of molecular medicine (Berlin, Germany) · 2023
    Article
  3. Article
  4. Perspectives in Genome-Editing Techniques for Livestock.Animals : an open access journal from MDPI · 2023
    Review
  5. Review
  6. Review
  7. Article
  8. Review
  9. Oscillometry of the respiratory system: a translational opportunity not to be missed.American journal of physiology. Lung cellular and molecular physiology · 2021
    Review
  10. IRE1α Is a Therapeutic Target for Cystic Fibrosis Airway Inflammation.International journal of molecular sciences · 2021
    Article
  11. Gene Therapy for Cystic Fibrosis: Lessons Learned and Paths Forward.Molecular therapy : the journal of the American Society of Gene Therapy · 2021
    Review
  12. Article
  13. Review
  14. The Building Blocks of Antimicrobial Resistance inFrontiers in cellular and infection microbiology · 2021
    Review
  15. Article
  16. Ivacaftor Reverses Airway Mucus Abnormalities in a Rat Model Harboring a Humanized G551D-CFTR.American journal of respiratory and critical care medicine · 2020
    Article
  17. Article
  18. Review
  19. Article
  20. Article

11 more citing papers are in PubMed but not listed here.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

2 authors.

Nicholas W KeiserDepartment of Anatomy and Cell Biology, University of Iowa Carver College of Medicine, Iowa City, Iowa 52242-1109, USA.
John F Engelhardt

Funding

Respiratory virus infection in early cystic fibrosis lung diseaseP01HL091842 · NHLBI · UNIVERSITY OF IOWA · PI ZABNER, JOSEPH · 2008 to 2022
$34.2M
Vector Core-Core 2P30DK054759 · NIDDK · UNIVERSITY OF IOWA · PI Amy Leanne Ryan · 1998 to 2026
$30.5M
Biology of submucosal gland stem cells in the airwayR37DK047967 · NIDDK · UNIVERSITY OF IOWA · PI ENGELHARDT, JOHN F · 2005 to 2014
$3.8M
MODELS OF SUBMUCOSAL GLAND DEVELOPMENT AND FUNCTIONR01DK047967 · NIDDK · UNIVERSITY OF PENNSYLVANIA · PI ENGELHARDT, JOHN F · 1993 to 2020
$3.8M
rAAV-directed gene therapy to the lung in cystic fibrosis ferret modelsR01HL108902 · NHLBI · UNIVERSITY OF IOWA · PI ENGELHARDT, JOHN F · 2011 to 2014
$2.2M
Pathological Evaluation of a Cystic Fibrosis Ferret ModelRC1HL099516 · NHLBI · UNIVERSITY OF IOWA · PI ENGELHARDT, JOHN F · 2009 to 2010
$999k
Etiology of Cystic Fibrosis-Related Diabetes in a CFTR-knockout FerretR24DK091211 · NIDDK · UNIVERSITY OF IOWA · PI APICELLA, MICHAEL A., COHEN, DAVID E. · 2011 to 2011
$485k
NHLBI NIH HHS HL091842NHLBI NIH HHS P01 HL091842NHLBI NIH HHS R01 HL108902NHLBI NIH HHS RC1 HL099516NHLBI NIH HHS RC1HL099516NIDDK NIH HHS DK047967NIDDK NIH HHS P30 DK054759NIDDK NIH HHS R01 DK047967NIDDK NIH HHS R24 DK091211NIDDK NIH HHS R24DK091211NIDDK NIH HHS R37 DK047967
6 · The paper itself

Abstract

purpose of reviewCystic fibrosis is the first human genetic disease to benefit from the directed engineering of three different species of animal models (mice, pigs, and ferrets). Recent studies on the cystic fibrosis pig and ferret models are providing new information about the pathophysiology of cystic fibrosis in various organ systems. Additionally, new conditional cystic fibrosis transmembrane conductance regulator (CFTR) knockout mice are teaching unexpected lessons about CFTR function in surprising cellular locations. Comparisons between these animal models and the human condition are key to dissecting the complexities of disease pathophysiology in cystic fibrosis. RECENT

findingsCystic fibrosis pigs and ferrets have provided new models to study the spontaneous development of disease in the lung and pancreas, two organs that are largely spared overt spontaneous disease in cystic fibrosis mice. New cystic fibrosis mouse models are now interrogating CFTR functions involved in growth and inflammation at an organ-based level using conditional knockout technology. Together, these models are providing new insights on the human condition. SUMMARY: Basic and clinical cystic fibrosis research will benefit greatly from the comparative pathophysiology of cystic fibrosis mice, pigs, and ferrets. Both similarities and differences between these three cystic fibrosis models will inform pathophysiologically important mechanisms of CFTR function in humans and aid in the development of both organ-specific and general therapies for cystic fibrosis.

Indexed as

Cystic Fibrosis Transmembrane Conductance RegulatorDisease Models, AnimalAnimalsCystic FibrosisFerretsHumansLungMicePancreasSwineCystic Fibrosis Transmembrane Conductance Regulator

Identifiers

PMID21857224
PMCPMC3596000

What OpenQuestion holds

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Registered trials

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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.