Assessment of Anti-α-Galactosidase A IgG Antibody Development in Fabry Disease Patients by ELISA and Purification of a Recombinant Human α-Galactosidase A from a Stable Overexpressing Human Cell Line
Bibliographic record
Abstract
Fabry disease is an X-linked lysosomal storage disorder caused by a deficiency in α-galactosidase A (α-gal A). Enzyme replacement therapy (ERT) consists of regular infusions of recombinant α-gal A. Fabry disease patients commonly develop IgG antibodies against this enzyme and there is a concern that they may reduce the efficacy and safety of ERT. We developed an ELISA to measure anti-α-gal A IgG antibodies and found that 61% of patients on ERT were seropositive. We also designed a fast, single-chromatography purification protocol for α-gal A from the supernatant of a human embryonic kidney 293T cell line engineered to stably overexpress a 6xhis-tagged human α-gal A. We obtained 0.342 mg/L with the expected molecular weight but lower enzymatic activity than expected. This α-gal A is bound by antibodies from seropositive patients and not by those from healthy individuals, which suggests that it may serve as antigen in our ELISA to measure anti-α-gal A antibodies.
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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 | 0.001 | 0.001 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.001 | 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 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".