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Record W4415708527 · doi:10.1126/science.ady7186

The functional landscape of coding variation in the familial hypercholesterolemia gene <i>LDLR</i>

2025· article· en· W4415708527 on OpenAlexaff
Daniel Tabet, Atina G. Coté, Megan Lancaster, Jochen Weile, Ashyad Rayhan, Iosifina Fotiadou, Nishka Kishore, Roujia Li, Da Kuang, Jennifer J. Knapp, Carmela Serio Carrero, Olivia Taverniti, Anna Axakova, Jack M.P. Castelli, Mohammad Majharul Islam, Shahin Sowlati‐Hashjin, Aanshi Gandhi, Ranim Maaieh, Michael Garton, Kenneth A. Matreyek, Douglas M. Fowler, Mafalda Bourbon, Simon G. Pfisterer, Andrew M. Glazer, Brett M. Kroncke, Victoria N. Parikh, Euan A. Ashley, Joshua W. Knowles, Melina Claussnitzer, Elizabeth T. Cirulli, Robert A. Hegele, Dan M. Roden, Calum A. MacRae, Frederick P. Roth

Bibliographic record

VenueScience · 2025
Typearticle
Languageen
FieldMedicine
TopicLipoproteins and Cardiovascular Health
Canadian institutionsWestern UniversityRobarts Clinical TrialsLunenfeld-Tanenbaum Research InstituteUniversity of Toronto
Fundersnot available
KeywordsFamilial hypercholesterolemiaMissense mutationHyperlipidemiaGenePhenotypeDiseaseLimitingLDL receptorMultifactorial Inheritance

Abstract

fetched live from OpenAlex

Variants in the familial hypercholesterolemia gene LDLR —the most important genetic driver of cardiovascular disease—can raise circulating low-density lipoprotein (LDL) cholesterol concentrations and increase the risk of premature atherosclerosis. Definitive classifications are lacking for nearly half of clinically encountered LDLR missense variants, limiting interventions that reduce disease burden. We tested the impact of ~17,000 (nearly all possible) LDLR coding variants on both LDLR cell-surface abundance and LDL uptake, yielding sequence–function maps that recapitulate known biochemistry, offer functional insights, and provide evidence for interpreting clinical variants. Functional scores correlated with hyperlipidemia phenotypes in prospective human cohorts and augmented polygenic scores to improve risk inference, highlighting the potential of this resource to accelerate familial hypercholesterolemia diagnosis and improve patient outcomes.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

How this classification was reachedexpand

Full frame machine prediction

Teacher imitation

Not 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.

metaresearch head score (Codex)0.001
metaresearch head score (Gemma)0.002
Version: metacan-v3-hybrid-931329e0061cValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: Observational
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.006
Threshold uncertainty score0.011

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0010.002
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0010.001
Science and technology studies0.0000.000
Scholarly communication0.0010.000
Open science0.0000.000
Research integrity0.0000.000
Insufficient payload (model declined to judge)0.0020.000

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.

Opus teacher head0.014
GPT teacher head0.265
Teacher spread0.250 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designObservational
Domainnot available
GenreEmpirical

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".

Quick stats

Citations21
Published2025
Admission routes1
Has abstractyes

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