ArticlePLoS genetics2009
Lack of CFTR in skeletal muscle predisposes to muscle wasting and diaphragm muscle pump failure in cystic fibrosis mice.
Article in PLoS genetics, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to 2 registered trials, which are not on this map. Cited by 52 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.
Cough Efficiency in Cystic Fibrosis
Effects of a Strength Exercise Program on Muscular Health in People With CF Treated With Modulators of the Cystic Fibrosis Transmembrane Conductance Regulator Receptor (CFTR)
Who cites it
52 citing papers in PubMed, 122 citations in OpenAlex.
- Exercise capacity and muscle fatiguability alterations following a progressive maximal exercise of lower extremities in children with cystic fibrosis.African health sciences · 2018Trial
- Article
- Impact of Elexacaftor-Tezacaftor-Ivacaftor on Muscle Composition in Cystic Fibrosis: An AI-Assisted Chest CT-Based Body Composition Analysis.Medical sciences (Basel, Switzerland) · 2025Observational
- Relationship between skeletal muscle indicators and clinical outcomes in cystic fibrosis is modified by adiposity and CFTR modulator use: a cross-sectional analysis.The American journal of clinical nutrition · 2025Article
- Strength-Oriented Virtual Exercise Training Intervention in Children and Adolescents with Cystic Fibrosis Under CFTR Modulators (the FIQMODE Study): Study Protocol for a Randomized Controlled Trial.Current protocols · 2025Article
- Potential therapeutic role of sex steroids in treating sarcopenia: a network pharmacology and molecular dynamics study.BMC pharmacology & toxicology · 2025Article
- Bone Disease in Cystic Fibrosis: Insights into Etiopathogenesis and Advances in Treatment Management.Journal of clinical medicine · 2025Review
- Respiratory Muscle Function in Children and Adolescents with Cystic Fibrosis in the Era of CFTR Modulator Therapies.Children (Basel, Switzerland) · 2025Article
- Dietary protein intake and overall diet quality in adults with cystic fibrosis following elexacaftor/tezacaftor/ivacaftor therapy.The British journal of nutrition · 2025Observational
- Peripheral Muscle Function and Body Composition in People With Cystic Fibrosis on Elexacaftor/Tezacaftor/Ivacaftor: A Cross-Sectional Single-Centre Study.Pediatric pulmonology · 2025Article
- Defective Cystic Fibrosis Transmembrane Conductance Regulator Accelerates Skeletal Muscle Aging by Impairing Autophagy/Myogenesis.Journal of cachexia, sarcopenia and muscle · 2025Article
- Article
- The long-term effect of elexacaftor/tezacaftor/ivacaftor on cardiorespiratory fitness in adolescent patients with cystic fibrosis: a pilot observational study.BMC pulmonary medicine · 2024Observational
- Computed tomography reveals hypertrophic remodelling of the diaphragm in cystic fibrosis but not in COPD.ERJ open research · 2023Article
- Skeletal muscle contributions to reduced fitness in cystic fibrosis youth.Frontiers in pediatrics · 2023Article
- Article
- Article
- Heart rate variability, exercise capacity and levels of daily physical activity in children and adolescents with mild-to-moderate cystic fibrosis.Hong Kong physiotherapy journal : official publication of the Hong Kong Physiotherapy Association Limited = Wu li chih liao · 2022Article
- Effects of a Short-Term Resistance-Training Program on Heart Rate Variability in Children With Cystic Fibrosis-A Randomized Controlled Trial.Frontiers in physiology · 2021Article
- Physical exercise as a tool to minimize the consequences of the Covid-19 quarantine: An overview for cystic fibrosis.Pediatric pulmonology · 2020Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
11 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Cystic fibrosis (CF) patients often have reduced mass and strength of skeletal muscles, including the diaphragm, the primary muscle of respiration. Here we show that lack of the CF transmembrane conductance regulator (CFTR) plays an intrinsic role in skeletal muscle atrophy and dysfunction. In normal murine and human skeletal muscle, CFTR is expressed and co-localized with sarcoplasmic reticulum-associated proteins. CFTR-deficient myotubes exhibit augmented levels of intracellular calcium after KCl-induced depolarization, and exposure to an inflammatory milieu induces excessive NF-kB translocation and cytokine/chemokine gene upregulation. To determine the effects of an inflammatory environment in vivo, sustained pulmonary infection with Pseudomonas aeruginosa was produced, and under these conditions diaphragmatic force-generating capacity is selectively reduced in Cftr(-/-) mice. This is associated with exaggerated pro-inflammatory cytokine expression as well as upregulation of the E3 ubiquitin ligases (MuRF1 and atrogin-1) involved in muscle atrophy. We conclude that an intrinsic alteration of function is linked to the absence of CFTR from skeletal muscle, leading to dysregulated calcium homeostasis, augmented inflammatory/atrophic gene expression signatures, and increased diaphragmatic weakness during pulmonary infection. These findings reveal a previously unrecognized role for CFTR in skeletal muscle function that may have major implications for the pathogenesis of cachexia and respiratory muscle pump failure in CF patients.
Indexed as
Identifiers
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.