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Record W4306932083 · doi:10.1016/j.xhgg.2022.100156

High-quality read-based phasing of cystic fibrosis cohort informs genetic understanding of disease modification

2022· article· en· W4306932083 on OpenAlexafffund
Scott Mastromatteo, Angela Chen, Jiafen Gong, Lin Fan, Bhooma Thiruvahindrapuram, Wilson W. L. Sung, J. Andrew Whitney, Zhuozhi Wang, Rohan Patel, Katherine Keenan, Anat Halevy, Naim Panjwani, Julie Avolio, Cheng Wang, Guillaume Côté-Maurais, Stéphanie Bégin, Damien Adam, Emmanuelle Brochiero, Candice Bjornson, Mark Chilvers, April Price, Michael D. Parkins, Richard van Wylick, Dimas Mateos‐Corral, D.J. Hughes, Mary Jane Smith, Nancy Morrison, Elizabeth Tullis, Anne L. Stephenson, Pearce Wilcox, Bradley S. Quon, Winnie M. Leung, Melinda Solomon, Lei Sun, Félix Ratjen, Lisa J. Strug

Bibliographic record

VenueHuman Genetics and Genomics Advances · 2022
Typearticle
Languageen
FieldMedicine
TopicCystic Fibrosis Research Advances
Canadian institutionsPublic Health OntarioUniversity of TorontoUniversity of Alberta HospitalAlberta Hospital EdmontonSt. Michael's HospitalQueen Elizabeth II Health Sciences CentreSt. Paul's HospitalAlberta Children's HospitalIzaak Walton Killam Health CentreLondon Health Sciences CentreHospital for Sick ChildrenUniversité de MontréalUniversity of CalgaryBC Children's HospitalCentre Hospitalier de l’Université de MontréalMemorial University of NewfoundlandSickKids FoundationKingston Health Sciences Centre
FundersNational Institute of Neurological Disorders and StrokeNational Cancer InstituteNational Human Genome Research InstituteNational Institute on Drug AbuseNational Institute of Mental HealthNational Heart, Lung, and Blood InstituteHospital for Sick ChildrenNational Institutes of HealthGovernment of CanadaCystic Fibrosis FoundationGovernment of OntarioNatural Sciences and Engineering Research Council of CanadaCystic Fibrosis CanadaCanadian Institutes of Health ResearchGenome Canada
KeywordsMeconium IleusHaplotypeGeneticsAlleleCystic fibrosisLocus (genetics)BiologyMissense mutationExome sequencingBioinformaticsGeneMeconiumMutationPregnancyFetus

Abstract

fetched live from OpenAlex

Phasing of heterozygous alleles is critical for interpretation of cis-effects of disease-relevant variation. We sequenced 477 individuals with cystic fibrosis (CF) using linked-read sequencing, which display an average phase block N50 of 4.39 Mb. We use these samples to construct a graph representation of CFTR haplotypes, demonstrating its utility for understanding complex CF alleles. These are visualized in a Web app, CFTbaRcodes, that enables interactive exploration of CFTR haplotypes present in this cohort. We perform fine-mapping and phasing of the chr7q35 trypsinogen locus associated with CF meconium ileus, an intestinal obstruction at birth associated with more severe CF outcomes and pancreatic disease. A 20-kb deletion polymorphism and a PRSS2 missense variant p.Thr8Ile (rs62473563) are shown to independently contribute to meconium ileus risk (p = 0.0028, p = 0.011, respectively) and are PRSS2 pancreas eQTLs (p = 9.5 × 10−7 and p = 1.4 × 10−4, respectively), suggesting the mechanism by which these polymorphisms contribute to CF. The phase information from linked reads provides a putative causal explanation for variation at a CF-relevant locus, which also has implications for the genetic basis of non-CF pancreatitis, to which this locus has been reported to contribute.

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 distilled prediction

Teacher imitation

Not 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.

metaresearch head score (Codex)0.000
metaresearch head score (Gemma)0.000
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: none
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.707
Threshold uncertainty score0.640

Codex and Gemma teacher scores by category

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

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 head0.048
GPT teacher head0.339
Teacher spread0.291 · 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 teacher head, 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

Citations8
Published2022
Admission routes2
Has abstractyes

Explore more

Same venueHuman Genetics and Genomics AdvancesSame topicCystic Fibrosis Research AdvancesFrench-language works237,207