Development and characterization of glial cell models of Autosomal Recessive Spastic Ataxia of Charle-voix-Saguenay
Bibliographic record
Abstract
"Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is the third most prevalent recessive ataxia, characterized by childhood onset of progressive cerebellar ataxia, spasticity, motor sensory neuropathy, axonal demyelination, and Purkinje cell loss. ARSACS is caused by loss-of- function mutations in the SACS gene, which encodes sacsin, a 520 kDa multimodular protein with a partially understood function. Several lines of evidence suggest that sacsin is involved in chaperone activities, promotes the proper polymerization of the neuronal intermediate filaments (i.e., neurofilaments and vimentin), is linked to mitochondrial dynamics, and bioenergetics and regulates organelle positioning by regulating cytoskeletal dynamics.(...)"
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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) | 0.001 | 0.001 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.002 | 0.002 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.001 |
| Open science | 0.001 | 0.000 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.000 | 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".