Comprehensive reanalysis for CNVs in ES data from unsolved rare disease cases results in new diagnoses.


Journal

NPJ genomic medicine
ISSN: 2056-7944
Titre abrégé: NPJ Genom Med
Pays: England
ID NLM: 101685193

Informations de publication

Date de publication:
26 Oct 2024
Historique:
received: 14 02 2024
accepted: 25 09 2024
medline: 27 10 2024
pubmed: 27 10 2024
entrez: 27 10 2024
Statut: epublish

Résumé

We report the results of a comprehensive copy number variant (CNV) reanalysis of 9171 exome sequencing datasets from 5757 families affected by a rare disease (RD). The data reanalysed was extremely heterogeneous, having been generated using 28 different enrichment kits by 42 different research groups across Europe partnering in the Solve-RD project. Each research group had previously undertaken their own analysis of the data but failed to identify disease-causing variants. We applied three CNV calling algorithms to maximise sensitivity, and rare CNVs overlapping genes of interest, provided by four partner European Reference Networks, were taken forward for interpretation by clinical experts. This reanalysis has resulted in a molecular diagnosis being provided to 51 families in this sample, with ClinCNV performing the best of the three algorithms. We also identified partially explanatory pathogenic CNVs in a further 34 individuals. This work illustrates the value of reanalysing ES cold cases for CNVs.

Identifiants

pubmed: 39461972
doi: 10.1038/s41525-024-00436-6
pii: 10.1038/s41525-024-00436-6
doi:

Types de publication

Journal Article

Langues

eng

Pagination

49

Investigateurs

Olaf Riess (O)
Tobias B Haack (TB)
Holm Graessner (H)
Birte Zurek (B)
Kornelia Ellwanger (K)
Marc Sturm (M)
Joohyun Park (J)
Leon Schütz (L)
Julia M Schulze-Hentrich (JM)
Rebecca Schüle (R)
Jishu Xu (J)
Melanie Kellner (M)
Baptist Resch (B)
Ingrid Kolen (I)
Matthis Synofzik (M)
Carlo Wilke (C)
Andreas Traschütz (A)
Danique Beijer (D)
Peter Heutink (P)
Ludger Schöls (L)
Holger Hengel (H)
Holger Lerche (H)
Christian Boßelmann (C)
Josua Kegele (J)
Robert Lauerer-Braun (R)
Stephan Lauxmann (S)
Han Brunner (H)
Hans Scheffer (H)
Nicoline Hoogerbrugge (N)
Peter A C 't Hoen (PAC)
Wouter Steyaert (W)
Richarda de Voer (R)
Erik-Jan Kamsteeg (EJ)
Bart van de Warrenburg (B)
Nienke van Os (N)
Iris Te Paske (IT)
Erik Janssen (E)
Elke de Boer (E)
Marloes Steehouwer (M)
Kornelia Neveling (K)
Bart van der Sanden (B)
Lydia Sagath (L)
Tjitske Kleefstra (T)
Anthony J Brookes (AJ)
Spencer Gibson (S)
Umar Riaz (U)
Greg Warren (G)
Sai Anuhya Nalagandla (SA)
Yunze Patrick Wang (YP)
Deepthi Sukumaran (D)
Sadegh Abadijou (S)
Volker Straub (V)
Chiara Marini Bettolo (CM)
Jordi Diaz Manera (JD)
Sophie Hambleton (S)
Karin Engelhardt (K)
Jill Clayton-Smith (J)
Siddharth Banka (S)
Elizabeth Alexander (E)
Adam Jackson (A)
Laurence Faivre (L)
Christel Thauvin (C)
Antonio Vitobello (A)
Anne-Sophie Denommé-Pichon (AS)
Yannis Duffourd (Y)
Ange-Line Bruel (AL)
Victor Couturier (V)
Ivo Glynne Gut (IG)
Davide Piscia (D)
Leslie Matalonga (L)
Anastasios Papakonstantinou (A)
Alberto Corvo (A)
Marcos Fernandez-Callejo (M)
Carles Hernández (C)
Daniel Picó (D)
Anna Esteve Codina (AE)
Marc Dabad (M)
Marta Gut (M)
Emanuele Raineri (E)
Gulcin Gumus (G)
Virginie Bros-Facer (V)
Ana Rath (A)
Marc Hanauer (M)
David Lagorce (D)
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Informations de copyright

© 2024. The Author(s).

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Auteurs

German Demidov (G)

Institute of Medical Genetics and Applied Genomics, University of Tübingen, Tübingen, Germany. German.Demidov@med.uni-tuebingen.de.
Institute for Bioinformatics and Medical Informatics (IBMI), University of Tübingen, Tübingen, Germany. German.Demidov@med.uni-tuebingen.de.

Burcu Yaldiz (B)

Department of Human Genetics, Radboud University Medical Center, Nijmegen, The Netherlands.
Department of Clinical Genetics, Maastricht University Medical Center, Maastricht, The Netherlands.

José Garcia-Pelaez (J)

i3S - Instituto de Investigação e Inovação em Saúde, Rua Alfredo Allen, 208, 4200-135, Porto, Portugal.
IPATIMUP - Institute of Molecular Pathology and Immunology, University of Porto, Porto, Portugal.
Faculty of Medicine, University of Porto, Porto, Portugal.

Elke de Boer (E)

Department of Human Genetics, Radboud University Medical Center, Nijmegen, The Netherlands.
Donders Institute for Brain, Cognition and Behaviour, Radboud University, Nijmegen, The Netherlands.
Department of Clinical Genetics, Erasmus MC University Medical Center, Rotterdam, The Netherlands.

Nika Schuermans (N)

Center for Medical Genetics, Ghent University Hospital, Ghent, Belgium.

Liedewei Van de Vondel (L)

Translational Neurosciences, Faculty of Medicine and Health Sciences, University of Antwerp, Antwerp, Belgium.
Laboratory of Neuromuscular Pathology, Institute Born-Bunge, University of Antwerp, Antwerp, Belgium.

Ida Paramonov (I)

Centro Nacional de Análisis Genómico (CNAG), C/Baldiri Reixac 4, 08028, Barcelona, Spain.
Universitat de Barcelona (UB), Barcelona, Spain.

Lennart F Johansson (LF)

University of Groningen, University Medical Center Groningen, Department of Genetics, Groningen, The Netherlands.

Francesco Musacchia (F)

Center for Human Technologies, Italian Institute of Technology (IIT), Genova, Italy.
Telethon Institute for Genetics and Medicine, 80078, Pozzuoli (Napoli), Italy.

Elisa Benetti (E)

Department of Medical Biotechnologies, Med Biotech Hub and Competence Center, University of Siena, 53100, Siena, Italy.

Gemma Bullich (G)

Centro Nacional de Análisis Genómico (CNAG), C/Baldiri Reixac 4, 08028, Barcelona, Spain.
Universitat de Barcelona (UB), Barcelona, Spain.

Karolis Sablauskas (K)

Department of Human Genetics, Radboud University Medical Center, Nijmegen, The Netherlands.
Institute of Data Science and Digital Technologies, Vilnius University, Vilnius, Lithuania.

Sergi Beltran (S)

Centro Nacional de Análisis Genómico (CNAG), C/Baldiri Reixac 4, 08028, Barcelona, Spain.
Universitat de Barcelona (UB), Barcelona, Spain.
Departament de Genètica, Microbiologia i Estadística, Facultat de Biologia, Universitat de Barcelona (UB), Barcelona, Spain.

Christian Gilissen (C)

Department of Human Genetics, Radboud University Medical Center, Nijmegen, The Netherlands.

Alexander Hoischen (A)

Department of Human Genetics, Radboud University Medical Center, Nijmegen, The Netherlands.
Radboud Institute for Molecular Life Sciences, Nijmegen, The Netherlands.
Department of Internal Medicine and Radboud Center for Infectious Diseases (RCI), Radboud University Medical Center, Nijmegen, The Netherlands.

Stephan Ossowski (S)

Institute of Medical Genetics and Applied Genomics, University of Tübingen, Tübingen, Germany.
Institute for Bioinformatics and Medical Informatics (IBMI), University of Tübingen, Tübingen, Germany.

Richarda de Voer (R)

Department of Human Genetics, Radboud University Medical Center, Nijmegen, The Netherlands.
Research Institute for Medical Innovation, Radboud University Medical Center, Nijmegen, The Netherlands.

Katja Lohmann (K)

Institute of Neurogenetics, University of Lübeck, Ratzeburger Allee 160, 23562, Lübeck, Germany.

Carla Oliveira (C)

i3S - Instituto de Investigação e Inovação em Saúde, Rua Alfredo Allen, 208, 4200-135, Porto, Portugal.
IPATIMUP - Institute of Molecular Pathology and Immunology, University of Porto, Porto, Portugal.
Faculty of Medicine, University of Porto, Porto, Portugal.

Ana Topf (A)

John Walton Muscular Dystrophy Research Centre, Translational and Clinical Research Institute, Newcastle University and Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK.

Lisenka E L M Vissers (LELM)

Department of Human Genetics, Radboud University Medical Center, Nijmegen, The Netherlands.
Donders Institute for Brain, Cognition and Behaviour, Radboud University, Nijmegen, The Netherlands.

Steven Laurie (S)

Centro Nacional de Análisis Genómico (CNAG), C/Baldiri Reixac 4, 08028, Barcelona, Spain. steven.laurie@cnag.eu.
Universitat de Barcelona (UB), Barcelona, Spain. steven.laurie@cnag.eu.

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