IGF system in children with congenital disorders of glycosylation
Bibliographic record
Abstract
OBJECTIVE: The function of IGF system components is affected by their glycosylation status in vitro. However, little is known about the role of glycosylation status of these components in vivo. In this study we determined the impact of glycosylation on the endocrine IGF system in children with the rare syndrome of congenital disorders of glycosylation (CDG). DESIGN: Analyses of serum samples from children with CDG and healthy controls. PATIENTS: Children with CDG (N = 12) were recruited as part of a separate clinical study of mannose therapy at the Mayo Clinic. Serum from control children (N = 11) were obtained as routine samples before discard. MEASUREMENTS: Levels and glycosylation state of components of the IGF system and ability to form physiologically relevant ternary complexes composed of IGF, IGFBP-3, and an acid-labile subunit (ALS). RESULTS: Serum levels of IGF-1, IGF-2, ALS, and IGFBP-3 were reduced (P < 0.05) in children with CDG when compared to controls. Immunoblot analysis showed incomplete glycosylation of ALS and IGFBP-3 and impaired ternary complex formation in CDG. Partial normalization of ALS and IGFBP-3 glycosylation was associated with improvement in linear growth in a child with CDG-Ib during initiation of oral mannose therapy. CONCLUSIONS: Inadequate glycosylation of IGFBP-3 and ALS has a negative effect on the function of these proteins in vivo. This study provides the first evidence in humans for the importance of glycosylation on components of the IGF system.
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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) | 0.000 | 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) | 0.000 | 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".