Dysfunctions of the Abdominal Aorta and Renal Arteries in a Mouse Model of Neurofibromatosis Type 1
Bibliographic record
Abstract
Neurofibromatosis 1 (NF1) is an autosomal dominant disorder presented with multiple café‐au‐lait spots, Lisch nodules,multiple nerve sheath tumors, and cardiovascular complications such as ischemia or aneurysm. Multiple vessels are affected in NF1 patients, but the abdominal aorta (AA) and renal arteries (RA) are most commonly reported, even though, the pathogenesis is not well understood. In this study, we examined functions of the AA and RA in a mouse model of NF1. We report a decrease in contraction and an increase in relaxation of smooth muscle cells (SMCs) in both the AA and RA at the age of 6 months. The nitric oxide synthase inhibitor (L‐NAME) completely blocked SMCs relaxation, indicating that the alterations resulted from enhanced nitric oxide (NO) production. The constitutional production of NO may reduce the availability of cofactors required for endothelial NOS to function. To determine if the increased production of NO had caused further dysfunction over time, we examined the AA in 9‐12 month old mice. Interestingly, we observed increased contraction and reduced relaxation in 9‐12 month old Nf1 +/‐ mice compared to controls, which may be indicative of a further decrease in NO bio‐availability. Taken together, our data demonstrates that underlying changes in SMCs and the endothelium contribute to vascular complications in NF1, providing new insights on potential future therapeutic approaches to address vascular complications in these patients. This study was funded by the British Columbia Neurofibromatosis Foundation and the Children's Tumor Foundation.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.000 |
| Meta-epidemiology (narrow) | 0.001 | 0.001 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.001 | 0.000 |
| Science and technology studies | 0.001 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.001 | 0.000 |
| Research integrity | 0.001 | 0.002 |
| Insufficient payload (model declined to judge) | 0.002 | 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 source (direct Gemma or distilled Codex), 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".