AMP‐activated protein kinase (AMPK) activity in Leigh Syndrome French Canadian Type (LSFC) patient fibroblasts: differences between primary and immortalized cell lines
Bibliographic record
Abstract
Background & Objective LSFC is an autosomal recessive disease caused by mutations in the leucine‐rich pentatricopeptide repeat containing protein (LRPPRC) gene resulting in decreased LRPPRC expression. This leads to a tissue‐specific cytochrome c oxidase deficiency and a reduction in energy production. AMPK is a key enzyme in the regulation of energy homeostasis. To test the hypothesis that AMPK activity may differ in LSFC and control cells, we evaluated the phosphorylated and active form of AMPK in primary (P) fibroblasts and, because of the limited availability of P cells, we also tested their immortalized (I) counterparts. Results Under basal conditions, P fibroblasts from LSFC patients and controls showed marginal differences in the levels of phosphorylated AMPK (pAMPK) as well as acetyl‐CoA carboxylase (ACC), one of many AMPK substrates. However, compared to P cells, I cells from both controls and LSFC patients showed a 60% and 80% decrease in pAMPK, respectively. Consistent with pAMPK levels, phosphorylated ACC levels were also decreased in I cells by 58 % in controls and 69% in LSFC patients. The total levels of these proteins were similar in all cases. Conclusion These results highlight an important decrease in AMPK activity level in I vs. P cells, which may compromise the capacity of these cells to palliate an energy deficit. Supported by: Association de l'acidose lactique and CIHR Emerging Team Grant
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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) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.003 | 0.001 |
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".