205. Functional Assessment of NSE-Cre Mouse Model of Friedreich Ataxia
Bibliographic record
Abstract
The characterization of mouse models of human disease is essential for understanding the underlying pathophysiology and developing new therapeutics. To understand more clearly the pathogenesis of sensorimotor dysfunction in Friedreich ataxia, we used conditional knockout mice in which the frataxin gene had been knocked-out in some tissues during embryogenesis by breeding them with mice expressing the Cre recombinase gene under the NSE promoter. The NSE-Cre mice grew poorly compared to their controls, and demonstrated a severe neurologic phenotype, and a shorter life span. They exhibited extended duration of the hindlimb clasping response, as well as slowed movement time on a suspended bar, severe ataxic gait and kyphosis. The mutants have poor somatosensory perception, breathing difficulties, heart palpitation and autonomic dysfunction as indicated by urogenital failure. Despite the short life span, the NSE-Cre mice are a valid model to evaluate behavioral deficits and could be used to test experimental therapies to improve functional responses.
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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 | 0.001 | 0.000 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.001 |
| Bibliometrics | 0.002 | 0.000 |
| Science and technology studies | 0.000 | 0.001 |
| Scholarly communication | 0.000 | 0.001 |
| Open science | 0.001 | 0.000 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.006 | 0.002 |
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".