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Record W4212829112 · doi:10.1038/s44161-022-00018-8

Extensive identification of genes involved in congenital and structural heart disorders and cardiomyopathy

2022· article· en· W4212829112 on OpenAlexafffund
Nadine Spielmann, Gregor Miller, Tudor I. Oprea, Chih‐Wei Hsu, Gisela Fobo, Goar Frishman, Corinna Montrone, Hamed Haseli Mashhadi, Jeremy Mason, Violeta Muñoz‐Fuentes, Stefanie Leuchtenberger, Andreas Ruepp, Matias Wagner, Dominik S. Westphal, Cordula M. Wolf, Agnes Görlach, Adrián Sanz‐Moreno, Yi-Li Cho, Raffaele Teperino, Stefan Brandmaier, Sapna Sharma, Isabella Galter, Manuela A. Östereicher, Lilly Zapf, Philipp Mayer‐Kuckuk, Jan Rozman, Lydia Teboul, Rosie Bunton-Stasyshyn, Heather Cater, Michelle Stewart, Skevoulla Christou, Henrik Westerberg, Amelia Willett, Janine M. Wotton, Willson Roper, Audrey E. Christiansen, Christopher Ward, Jason D. Heaney, Corey Reynolds, Jan Procházka, Lynette Bower, David Clary, Mohammed Selloum, Ghina Bou About, Olivia Wendling, Hugues Jacobs, Sophie Leblanc, Hamid Méziane, Tania Sorg, Enrique Audain, Arthur Gilly, Nigel W. Rayner, Juan Antonio Aguilar‐Pimentel, Lore Becker, Lillian Garrett, Sabine M. Hölter, Oana V. Amarie, Julia Calzada‐Wack, Tanja Klein‐Rodewald, Patricia da Silva‐Buttkus, Christoph Lengger, Claudia Stoeger, Raffaele Gerlini, Birgit Rathkolb, Daniela Mayr, John R. Seavitt, Angelina Gaspero, Jennie R. Green, Arturo Garza, Ritu Bohat, Leeyean Wong, Melissa L. McElwee, Sowmya Kalaga, Tara L. Rasmussen, Isabel Lorenzo, Denise G. Lanza, Rodney C. Samaco, Surabi Veeraragaven, Juan Gallegos, Petr Kašpárek, Silvia Petrezsélyová, Ruairidh King, Sara Johnson, James Cleak, Zsombor Szkoe-Kovacs, Gemma Codner, Matthew Mackenzie, Adam Caulder, Janet Kenyon, Wendy Gardiner, Hayley Phelps, Rhys Hancock, Claire Norris, Michayla Moore, Audrie Seluke, Rachel Urban, Coleen Kane, Leslie O. Goodwin, Kevin A. Peterson, Matthew Mckay, Jenn J. Cook, Jacob P. Lowy, Michael McFarland, Joshua A. Wood, Brandon Willis, Heather Tolentino, Todd Tolentino, Michael Schuchbauer, Jason Salazar, Jennifer Johnson, Rebecca Munson, Abdel Ayadi, Guillaume Pavlovic, Marie‐Christine Birling, Sylvie Jacquot, Dalila Ali-Hadji, Philippe Charles, Philippe André, Marie‐France Champy, Fabrice Riet, Igor Vukobradovic, Zorana Berberovic, Dawei Qu, Ruolin Guo, Abigail D’Souza, Ziyue Huang, Susan Camilleri, Milan Ganguly, Hibret A. Adissu, Mohammed Eskandarian, Xueyuan Shang, Kyle Duffin, Catherine K. Xu, Kyle Roberton, Valerie Laurin, Qing Lan, Gillian Sleep, Amie Creighton, Lauri G. Lintott, Marina Gertsenstein, Monica Pereira, Sandra Tondat, Amit Patel, Maribelle Cruz, Alex Bezginov, D. Craig Miller, Wang Hy, Atsushi Yoshiki, Nobuhiko Tanaka, Masaru Tamura, Zhiwei Liu, Olga Ermakova, Paolo Fruscoloni, Claudia Seisenberger, Antje Bürger, Florian Giesert, John C. Ambrose, P. Arumu gam, R. Bevers, Marta Bleda, C. R. Boustred, Helen Brittain, Mark J. Caulfield, Gcf Chan, Tom Fowler, Adam Giess, Angela Hamblin, Shirley Henderson, Tim Hubbard, Robert W. Jackson, J. Louise Jones, Dalia Kasperavičiūtė, Melis Kayikci, Athanasios Kousathanas, L. Lahnstein, S. E. A. Leigh, I. U. S. Leong, F. J. Lopez, F. Maleady-Crowe, Meriel McEntagart, Federico Minneci, Loukas Moutsianas, M. Mueller, Nirupa Murugaesu, Anna C. Need, Peter O’Donovan, Chris A. Odhams, Christine Patch, D. Perez-Gil, J. Pullinger, T. Rahim, Augusto Rendon, T. Rogers, K. Savage, K. Sawant, Richard H. Scott, Afshan Siddiq, A. Sieghart, S. C. Smith, Alona Sosinsky, Alexander Stuckey, M. Tanguy, A. L. Taylor-Tavares, Ellen Thomas, Simon R. Thompson, Arianna Tucci, M. J. Welland, Eleanor Williams, Katarzyna Witkowska, S. M. Wood, Marc‐Phillip Hitz, Eleftheria Zeggini, Eckhard Wolf, Radislav Sedláček, Steven A. Murray, Karen L. Svenson, Robert E. Braun, Jaqueline K. White, Lois Kelsey, Xiang Gao, Toshihiko Shiroishi, Ying Xu, Je Kyung Seong, Fabio Mammano, Glauco P. Tocchini‐Valentini, Arthur L. Beaudet, Helen Parkinson, Damian Smedley, Ann‐Marie Mallon, Sara Wells, Harald Grallert, Wolfgang Wurst, Susan Marschall, Helmut Fuchs, Steve D. M. Brown, Ann M. Flenniken, Lauryl M. J. Nutter, Colin McKerlie, Yann Hérault, K. C. Kent Lloyd, Mary E. Dickinson, Valérie Gailus‐Durner, Martin Hrabě de Angelis

Bibliographic record

VenueNature Cardiovascular Research · 2022
Typearticle
Languageen
FieldBiochemistry, Genetics and Molecular Biology
TopicCongenital heart defects research
Canadian institutionsLunenfeld-Tanenbaum Research InstituteHospital for Sick ChildrenSinai Health SystemToronto Centre for Phenogenomics
FundersNational Heart, Lung, and Blood InstituteCzech Centre for Phenogenomics, Institute of Molecular Genetics of the Czech Academy of SciencesHelmholtz Zentrum MünchenMedical Research CouncilCentre National de la Recherche ScientifiquePHENOMINSoochow UniversityUniversité de StrasbourgInstitut National de la Santé et de la Recherche MédicaleEuropean CommissionNational Human Genome Research InstituteAgence Nationale de la RechercheU.S. Department of Health and Human ServicesNational Institutes of HealthOntario GenomicsGenome Canada
KeywordsHeart diseaseCardiomyopathyCardiac function curveDiseaseMedicineInternal medicineCardiologyHeart failure

Abstract

fetched live from OpenAlex

Clinical presentation of congenital heart disease is heterogeneous, making identification of the disease-causing genes and their genetic pathways and mechanisms of action challenging. By using in vivo electrocardiography, transthoracic echocardiography and microcomputed tomography imaging to screen 3,894 single-gene-null mouse lines for structural and functional cardiac abnormalities, here we identify 705 lines with cardiac arrhythmia, myocardial hypertrophy and/or ventricular dilation. Among these 705 genes, 486 have not been previously associated with cardiac dysfunction in humans, and some of them represent variants of unknown relevance (VUR). Mice with mutations in Casz1, Dnajc18, Pde4dip, Rnf38 or Tmem161b genes show developmental cardiac structural abnormalities, with their human orthologs being categorized as VUR. Using UK Biobank data, we validate the importance of the DNAJC18 gene for cardiac homeostasis by showing that its loss of function is associated with altered left ventricular systolic function. Our results identify hundreds of previously unappreciated genes with potential function in congenital heart disease and suggest causal function of five VUR in congenital heart disease.

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)0.000
metaresearch head score (Gemma)0.001
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: Empirical
Teacher disagreement score0.004
Threshold uncertainty score0.013

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0000.001
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0010.001
Bibliometrics0.0020.002
Science and technology studies0.0000.000
Scholarly communication0.0000.000
Open science0.0000.001
Research integrity0.0000.000
Insufficient payload (model declined to judge)0.0040.001

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.

Opus teacher head0.015
GPT teacher head0.313
Teacher spread0.298 · 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

Citations68
Published2022
Admission routes2
Has abstractyes

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