Molecular Pathophysiology of Myofiber Injury in Deficiencies of the Dystrophin-Glycoprotein Complex
Bibliographic record
Abstract
Duchenne muscular dystrophy is caused by mutations in the gene encoding dystrophin, a 427 kd protein normally found at the cytoplasmic face of the sarcolemma. In normal muscle, dystrophin is associated with a multimolecular glycoprotein complex. Primary mutations in the genes encoding members of this glycoprotein complex are also associated with muscular dystrophy. The dystrophin-glycoprotein complex provides a physical linkage between the internal cytoskeleton of myofibers and the extracellular matrix, but the precise functions of the dystrophin-glycoprotein complex remain uncertain. In this review, five potential pathogenetic mechanisms implicated in the initiation of myofiber injury in dystrophin-glycoprotein complex deficiencies are discussed: (1) mechanical weakening of the sarcolemma, (2) inappropriate calcium influx, (3) aberrant cell signaling, (4) increased oxidative stress, and (5) recurrent muscle ischemia. Particular emphasis is placed on the multifunctional nature of the dystrophin-glycoprotein complex and the fact that the above mechanisms are in no way mutually exclusive and may interact with one another to a significant degree.
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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 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.001 | 0.000 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.002 | 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".