Neuromuscular dysfunction in the mutant superoxide dismutase mouse model of amyotrophic lateral sclerosis
Why this work is in the frame
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Bibliographic record
Abstract
To better understand the interaction between motor neuron dysfunction and denervation in amyotrophic lateral sclerosis (ALS), we have evaluated motor neuron number and the retrograde uptake and transport of fluorogold by motor neurons in mice overexpressing mutant superoxide dismutase (mSOD), and wild-type controls. N-CAM immunoreactivity and protein kinase expression were determined in skeletal muscle during denervation. We found that in severely affected mSOD mice, motor neuron loss is moderate (approximate 40% reduction), whereas retrograde uptake/transport as assessed using fluorogold is profoundly impaired (approximately 90% reduction). The impairment in fluorogold uptake/transport corresponds to measures of progressive muscle denervation such as increased N-CAM immunoreactivity of muscle and increased expression of protein kinase B (PKB) in denervated muscle. These data suggest that the debility in the mSOD mouse model of ALS is produced, in part, by impaired retrograde uptake/transport in motor neuron axons in spite of regenerative support from muscle such as elevated expression of PKB.
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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 | 0.001 | 0.000 |
| Meta-epidemiology (narrow) | 0.001 | 0.001 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.001 | 0.002 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.000 | 0.001 |
| Open science | 0.001 | 0.000 |
| Research integrity | 0.001 | 0.002 |
| 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 it