Espacio público europeo y patriotismo constitucional en Habermas
Bibliographic record
Abstract
The <i>RLBP1</i> gene encodes the 36 kDa cellular retinaldehyde-binding protein, CRALBP, a soluble retinoid carrier, in the visual cycle of the eyes. Mutations in <i>RLBP1</i> are associated with recessively inherited clinical phenotypes, including Bothnia dystrophy, retinitis pigmentosa, retinitis punctata albescens, fundus albipunctatus, and Newfoundland rod-cone dystrophy. However, the etiology of these retinal disorders is not well understood. Here, we generated homologous zebrafish models to bridge this knowledge gap. Duplication of the <i>rlbp1</i> gene in zebrafish and cell-specific expression of the paralogs <i>rlbp1a</i> in the retinal pigment epithelium and <i>rlbp1b</i> in Müller glial cells allowed us to create intrinsically cell type-specific knockout fish lines. Using <i>rlbp1a</i> and <i>rlbp1b</i> single and double mutants, we investigated the pathological effects on visual function. Our analyses revealed that <i>rlbp1a</i> was essential for cone photoreceptor function and chromophore metabolism in the fish eyes. <i>rlbp1a-</i>mutant fish displayed reduced chromophore levels and attenuated cone photoreceptor responses to light stimuli. They accumulated 11-<i>cis</i> and all-<i>trans</i>-retinyl esters which displayed as enlarged lipid droplets in the RPE reminiscent of the subretinal yellow-white lesions in patients with <i>RLBP1</i> mutations. During aging, these fish developed retinal thinning and cone and rod photoreceptor dystrophy. In contrast, <i>rlbp1b</i> mutants did not display impaired vision. The double mutant essentially replicated the phenotype of the <i>rlbp1a</i> single mutant. Together, our study showed that the <i>rlbp1a</i> zebrafish mutant recapitulated many features of human blinding diseases caused by <i>RLBP1</i> mutations and provided novel insights into the pathways for chromophore regeneration of cone photoreceptors.
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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 | 0.001 | 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 | 0.001 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.001 | 0.000 |
| Research integrity | 0.000 | 0.001 |
| Insufficient payload (model declined to judge) | 0.006 | 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; both teacher heads agree on what is shown here.
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".