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Record W2947227562 · doi:10.1002/humu.23818

Large scale multifactorial likelihood quantitative analysis of <i>BRCA1</i> and <i>BRCA2</i> variants: An ENIGMA resource to support clinical variant classification

2019· article· en· W2947227562 on OpenAlexafffund
Michael T. Parsons, Emma Tudini, Hongyan Li, Eric Hahnen, Barbara Wappenschmidt, Lídia Feliubadaló, Cora M. Aalfs, Simona Agata, Kristiina Aittomäki, Elisa Alducci, María Concepción Alonso‐Cerezo, Norbert Arnold, Bernd Auber, Rachel Austin, Jacopo Azzollini, Judith Balmañà, Elena Barbieri, Claus R. Bartram, Ana Blanco, Britta Blümcke, Sandra Bonache, Bernardo Bonanni, Åke Borg, Beatrice Bortesi, Joan Brunet, Carla Bruzzone, Karolin Bucksch, Giulia Cagnoli, Trinidad Caldés, Almuth Caliebe, Maria A. Caligo, Mariarosaria Calvello, Gabriele Lorenzo Capone, Sandrine M. Caputo, Ileana Carnevali, Estela Carrasco, Virginie Caux‐Moncoutier, Pietro Cavalli, Giulia Cini, Edward Clarke, Paola Concolino, Elisa J. Cops, Laura Cortesi, Fergus J. Couch, Esther Darder, Miguel de la Hoya, Michael Dean, Irmgard Debatin, Jesús Del Valle, Capucine Delnatte, Nicolas Derive, Orland Dı́ez, Nina Ditsch, Susan M. Domchek, Véronique Dutrannoy, Diana Eccles, Hans Ehrencrona, Ute Enders, D. Gareth Evans, Chantal Farra, Ulrike Faust, Ute Felbor, Irène Feroce, Miriam Fine, William D. Foulkes, Henrique C.R. Galvão, Gaetana Gambino, Andrea Gehrig, Francesca Gensini, Anne‐Marie Gerdes, Aldo Germani, J Giesecke, Viviana Gismondi, Carolina Gómez, E. Gómez, Sara González, Èlia Grau, Sabine Grill, Eva Groß, Aliana Guerrieri‐Gonzaga, Marine Guillaud‐Bataille, Sara Gutiérrez‐Enríquez, Thomas Haaf, Karl Hackmann, Thomas van Overeem Hansen, Marion Harris, Jan Hauke, T. Heinrich, Heide Hellebrand, Karen Herold, Ellen Honisch, Judit Horváth, Claude Houdayer, Verena Hübbel, Sílvia Iglesias, Á. Izquierdo, Paul A. James, Linda A.M. Janssen, Udo Jeschke, Silke Kaulfuß, Katharina Keupp, Marion Kiechle, Alexandra C. Kölbl, Sophie Krieger, Torben A. Kruse, Anders Kvist, Fiona Lalloo, Mirjam Larsen, Vanessa Lattimore, Charlotte Kvist Lautrup, Susanne Ledig, Elena Leinert, Alexandra Lewis, Joanna Lim, Markus Loeffler, Adrià López‐Fernández, Emanuela Lucci‐Cordisco, Nicolai Maass, Siranoush Manoukian, Monica Marabelli, Laura Matricardi, Alfons Meindl, Rodrigo D. Michelli, Setareh Moghadasi, Alejandro Moles‐Fernández, Marco Montagna, Gemma Montalban, Álvaro N.A. Monteiro, Eva Montes, Luigi Mori, Lidia Moserle, Clemens R. Müller, Christoph Mundhenke, Nadia Naldi, Katherine L. Nathanson, Matilde Navarro, Heli Nevanlinna, Cassandra Nichols, Dieter Niederacher, Henriette Roed Nielsen, Kai‐Ren Ong, Nicholas Pachter, Edenir Inêz Palmero, Laura Papi, Inge Søkilde Pedersen, Bernard Peissel, Pedro Pérez‐Segura, Katharina Pfeifer, Marta Pineda, Esther Pohl‐Rescigno, Nicola Poplawski, Berardino Porfirio, Anne S. Quante, Juliane Ramser, Rui Manuel Reis, Françoise Révillion, Kerstin Rhiem, Barbara Riboli, Julia Ritter, Daniela Rivera, Paula Rofes, Andreas Rump, Mónica Salinas, A.M. SÁnchez De Abajo, Gunnar Schmidt, Ulrike Schoenwiese, Jochen Seggewiß, Ares Solanes, Doris Steinemann, Mathias Stiller, Dominique Stoppa‐Lyonnet, Kelly J. Sullivan, Rachel Susman, Christian Sutter, Sean V. Tavtigian, Soo‐Hwang Teo, Àlex Teulé, Mads Thomassen, Maria Grazia Tibiletti, Marc Tischkowitz, Silvia Tognazzo, Amanda E. Toland, Eva Tornero, Therese Törngren, Sara Torres‐Esquius, Angela Toss, Alison Trainer, Kathy Tucker, Christi J. van Asperen, Marion van Mackelenbergh, Liliana Varesco, Gardenia Vargas‐Parra, Raymonda Varon, Ana Vega, Ángela Velasco, Anne‐Sophie Vesper, Alessandra Viel, Maaike P.G. Vreeswijk, Sebastian Wagner, Anke Waha, Logan C. Walker, Rhiannon Walters, Shan Wang‐Gohrke, Bernhard Weber, Wilko Weichert, Kerstin Wieland, Lisa Wiesmüller, Isabell Witzel, Achim Wöckel, Emma R. Woodward, Silke Zachariae, Valentina Zampiga, C Zeder-Göß, KConFab Investigators, Conxi Lázaro, Arcangela De Nicolo, Paolo Radice, Christoph Engel, Rita K. Schmutzler, David E. Goldgar, Amanda B. Spurdle

Bibliographic record

VenueHuman Mutation · 2019
Typearticle
Languageen
FieldBiochemistry, Genetics and Molecular Biology
TopicBRCA gene mutations in cancer
Canadian institutionsMcGill University
FundersNational Cancer InstituteInternational Graduate School in Molecular Medicine UlmCancer Council TasmaniaCancer Council NSWCancer Council South AustraliaManchester Biomedical Research CentreNational Institutes of HealthSeventh Framework ProgrammeCentro de Investigación Biomédica en Red de CáncerCancer Council VictoriaDeutsche KrebshilfeAssociazione Italiana per la Ricerca sul CancroFinanciadora de Estudos e ProjetosMax-Planck-GesellschaftConselho Nacional de Desenvolvimento Científico e TecnológicoUniversität UlmUniversiteit LeidenUnicancerNewcastle UniversityRoyal Society Te ApārangiNational Breast Cancer FoundationWellcome TrustCancer Research UKCancer AustraliaHuntsman Cancer InstituteNederlandse Organisatie voor Wetenschappelijk OnderzoekEuropean CommissionBreast Cancer Research FoundationCanadian Institutes of Health ResearchFundación Mutua MadrileñaGeneralitat de CatalunyaMedical Research CouncilFondazione PisaNational Institute for Health and Care ResearchInstituto de Salud Carlos IIIOhio State UniversityHospital de Câncer de BarretosQIMR Berghofer Medical Research InstituteNational Health and Medical Research CouncilAstraZeneca
KeywordsBiologyPathogenicityGeneticsMissense mutationComputational biologyRNA splicingGenePopulationBioinformaticsMutationMedicine

Abstract

fetched live from OpenAlex

The multifactorial likelihood analysis method has demonstrated utility for quantitative assessment of variant pathogenicity for multiple cancer syndrome genes. Independent data types currently incorporated in the model for assessing BRCA1 and BRCA2 variants include clinically calibrated prior probability of pathogenicity based on variant location and bioinformatic prediction of variant effect, co-segregation, family cancer history profile, co-occurrence with a pathogenic variant in the same gene, breast tumor pathology, and case-control information. Research and clinical data for multifactorial likelihood analysis were collated for 1,395 BRCA1/2 predominantly intronic and missense variants, enabling classification based on posterior probability of pathogenicity for 734 variants: 447 variants were classified as (likely) benign, and 94 as (likely) pathogenic; and 248 classifications were new or considerably altered relative to ClinVar submissions. Classifications were compared with information not yet included in the likelihood model, and evidence strengths aligned to those recommended for ACMG/AMP classification codes. Altered mRNA splicing or function relative to known nonpathogenic variant controls were moderately to strongly predictive of variant pathogenicity. Variant absence in population datasets provided supporting evidence for variant pathogenicity. These findings have direct relevance for BRCA1 and BRCA2 variant evaluation, and justify the need for gene-specific calibration of evidence types used for variant classification.

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)0.020
metaresearch head score (Gemma)0.075
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: none
GenreCandidate signal: Empirical · Consensus signal: none
Teacher disagreement score0.020
Threshold uncertainty score0.107

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0200.075
Meta-epidemiology (narrow)0.0010.001
Meta-epidemiology (broad)0.0020.002
Bibliometrics0.0100.006
Science and technology studies0.0010.001
Scholarly communication0.0050.002
Open science0.0020.004
Research integrity0.0010.002
Insufficient payload (model declined to judge)0.0090.004

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 head0.032
GPT teacher head0.357
Teacher spread0.324 · 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".

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Citations152
Published2019
Admission routes2
Has abstractyes

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