The Role of Cystic Fibrosis Transmembrane Conductance Regulator in Skeletal Muscle Contractile Function
Bibliographic record
Abstract
Purpose: Genetic mutations in cystic fibrosis (CF) result in CF transmembrane conductance regulator (CFTR) dysfunction. CFTR is expressed in human skeletal muscle; its effect on skeletal muscle abnormalities is unknown. The study objective is to investigate the role of CFTR in skeletal muscle contractile function. Methods: We conducted a prospective, cross-sectional study comparing 34 adults with minimal and 18 with residual function CFTR mutations, recruited from Toronto Adult CF Centre, St. Michael's Hospital, Unity Health Toronto. Quadriceps, biceps brachii, and handgrip strength was measured with dynamometers; leg muscle power with the stair climb power test. Quadriceps muscle contractility was determined by quadriceps muscle strength normalized to quadriceps muscle size, measured with ultrasound images. Multivariable regression was used for analysis. Results: People with residual function CFTR mutations had higher quadriceps muscle torque normalized to quadriceps layer thickness and to rectus femoris cross-sectional area by 27.5 Nm/cm [95% CI (2.2, 52.8) Nm/cm, P = .034] and 5.6 Nm/cm 2 [95% CI (0.3, 10.9) Nm/cm 2 , P = .041], respectively, compared with those with minimal function CFTR mutations. There were no differences in quadriceps muscle torque ( P = .58), leg muscle power ( P = .47), biceps brachii muscle force ( P = .14), or handgrip force ( P = .12) between the 2 mutation groups. Conclusions: CFTR protein may play a role in muscle contractility, implying a limited capacity to exert muscle force per unit of muscle size in people with CF. This suggests that building a greater muscle mass through resistance exercises focusing on muscle hypertrophy in exercise prescription may improve muscle strength in people with CF.
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How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.000 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.001 |
| Insufficient payload (model declined to judge) | 0.000 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one teacher head, not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".