Whole genome sequencing delineates regulatory, copy number, and cryptic splice variants in early onset cardiomyopathy.


Journal

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

Informations de publication

Date de publication:
14 Mar 2022
Historique:
received: 10 08 2021
accepted: 04 02 2022
entrez: 15 3 2022
pubmed: 16 3 2022
medline: 16 3 2022
Statut: epublish

Résumé

Cardiomyopathy (CMP) is a heritable disorder. Over 50% of cases are gene-elusive on clinical gene panel testing. The contribution of variants in non-coding DNA elements that result in cryptic splicing and regulate gene expression has not been explored. We analyzed whole-genome sequencing (WGS) data in a discovery cohort of 209 pediatric CMP patients and 1953 independent replication genomes and exomes. We searched for protein-coding variants, and non-coding variants predicted to affect the function or expression of genes. Thirty-nine percent of cases harbored pathogenic coding variants in known CMP genes, and 5% harbored high-risk loss-of-function (LoF) variants in additional candidate CMP genes. Fifteen percent harbored high-risk regulatory variants in promoters and enhancers of CMP genes (odds ratio 2.25, p = 6.70 × 10

Identifiants

pubmed: 35288587
doi: 10.1038/s41525-022-00288-y
pii: 10.1038/s41525-022-00288-y
pmc: PMC8921194
doi:

Types de publication

Journal Article

Langues

eng

Pagination

18

Subventions

Organisme : Medical Research Council
ID : MC_EX_MR/M009203/1
Pays : United Kingdom
Organisme : Medical Research Council
ID : MR/M009203/1
Pays : United Kingdom
Organisme : Wellcome Trust
Pays : United Kingdom
Organisme : Medical Research Council
ID : MC_PC_14089
Pays : United Kingdom

Investigateurs

J C Ambrose (JC)
P Arumugam (P)
E L Baple (EL)
M Bleda (M)
F Boardman-Pretty (F)
J M Boissiere (JM)
C R Boustred (CR)
H Brittain (H)
M J Caulfield (MJ)
G C Chan (GC)
C E H Craig (CEH)
L C Daugherty (LC)
A de Burca (A)
A Devereau (A)
G Elgar (G)
R E Foulger (RE)
T Fowler (T)
P Furió-Tarí (P)
A Giess (A)
J M Hackett (JM)
D Halai (D)
A Hamblin (A)
S Henderson (S)
J E Holman (JE)
T J P Hubbard (TJP)
K Ibáñez (K)
R Jackson (R)
L J Jones (LJ)
D Kasperaviciute (D)
M Kayikci (M)
A Kousathanas (A)
L Lahnstein (L)
K Lawson (K)
S E A Leigh (SEA)
I U S Leong (IUS)
F J Lopez (FJ)
F Maleady-Crowe (F)
J Mason (J)
E M McDonagh (EM)
L Moutsianas (L)
M Mueller (M)
N Murugaesu (N)
A C Need (AC)
C A Odhams (CA)
A Orioli (A)
C Patch (C)
D Perez-Gil (D)
M B Pereira (MB)
D Polychronopoulos (D)
J Pullinger (J)
T Rahim (T)
A Rendon (A)
P Riesgo-Ferreiro (P)
T Rogers (T)
M Ryten (M)
K Savage (K)
K Sawant (K)
R H Scott (RH)
A Siddiq (A)
A Sieghart (A)
D Smedley (D)
K R Smith (KR)
S C Smith (SC)
A Sosinsky (A)
W Spooner (W)
H E Stevens (HE)
A Stuckey (A)
R Sultana (R)
M Tanguy (M)
E R A Thomas (ERA)
S R Thompson (SR)
C Tregidgo (C)
A Tucci (A)
E Walsh (E)
S A Watters (SA)
M J Welland (MJ)
E Williams (E)
K Witkowska (K)
S M Wood (SM)
M Zarowiecki (M)

Informations de copyright

© 2022. The Author(s).

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Auteurs

Robert Lesurf (R)

Genetics and Genome Biology Program, The Hospital for Sick Children, Toronto, ON, Canada.

Abdelrahman Said (A)

Genetics and Genome Biology Program, The Hospital for Sick Children, Toronto, ON, Canada.

Oyediran Akinrinade (O)

Genetics and Genome Biology Program, The Hospital for Sick Children, Toronto, ON, Canada.
St. George's University School of Medicine, Grenada, Grenada.

Jeroen Breckpot (J)

Department of Human Genetics, UZ Leuven, Leuven, Belgium.

Kathleen Delfosse (K)

Genetics and Genome Biology Program, The Hospital for Sick Children, Toronto, ON, Canada.

Ting Liu (T)

Genetics and Genome Biology Program, The Hospital for Sick Children, Toronto, ON, Canada.

Roderick Yao (R)

Genetics and Genome Biology Program, The Hospital for Sick Children, Toronto, ON, Canada.

Gabrielle Persad (G)

Genetics and Genome Biology Program, The Hospital for Sick Children, Toronto, ON, Canada.
Department of Molecular Genetics, University of Toronto, Toronto, ON, Canada.

Fintan McKenna (F)

Genetics and Genome Biology Program, The Hospital for Sick Children, Toronto, ON, Canada.

Ramil R Noche (RR)

Genetics and Genome Biology Program, The Hospital for Sick Children, Toronto, ON, Canada.
Zebrafish Genetics and Disease Models Core, The Hospital for Sick Children, Toronto, ON, Canada.

Winona Oliveros (W)

Life Sciences Department, Barcelona Supercomputing Center, Barcelona, Catalonia, Spain.

Kaia Mattioli (K)

Division of Genetics, Department of Medicine, Brigham & Women's Hospital and Harvard Medical School, Boston, MA, USA.

Shreya Shah (S)

Genetics and Genome Biology Program, The Hospital for Sick Children, Toronto, ON, Canada.

Anastasia Miron (A)

Genetics and Genome Biology Program, The Hospital for Sick Children, Toronto, ON, Canada.

Qian Yang (Q)

Genetics and Genome Biology Program, The Hospital for Sick Children, Toronto, ON, Canada.

Guoliang Meng (G)

Developmental and Stem Cell Biology Program, The Hospital for Sick Children, Toronto, ON, Canada.

Michelle Chan Seng Yue (MCS)

Princess Margaret Cancer Center, University Health Network, Toronto, ON, Canada.

Wilson W L Sung (WWL)

The Centre for Applied Genomics, The Hospital for Sick Children, Toronto, ON, Canada.

Bhooma Thiruvahindrapuram (B)

The Centre for Applied Genomics, The Hospital for Sick Children, Toronto, ON, Canada.

Jane Lougheed (J)

Division of Cardiology, Children's Hospital of Eastern Ontario, Ottawa, ON, Canada.

Erwin Oechslin (E)

Peter Munk Cardiac Centre, Division of Cardiology, Toronto General Hospital, University of Toronto, Toronto, ON, Canada.

Tapas Mondal (T)

Department of Pediatrics, Hamilton Health Sciences Centre, Hamilton, ON, Canada.

Lynn Bergin (L)

Division of Cardiology, London Health Sciences Centre, London, ON, Canada.

John Smythe (J)

Department of Pediatrics, Kingston General Hospital, Kingston, ON, Canada.

Shashank Jayappa (S)

Cardiovascular Biology and Disease Theme, Institute for Stem Cell Science and Regenerative Medicine, Bangalore (inStem), Bangalore, India.

Vinay J Rao (VJ)

Cardiovascular Biology and Disease Theme, Institute for Stem Cell Science and Regenerative Medicine, Bangalore (inStem), Bangalore, India.

Jayaprakash Shenthar (J)

Department of Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bengaluru, India.

Perundurai S Dhandapany (PS)

Cardiovascular Biology and Disease Theme, Institute for Stem Cell Science and Regenerative Medicine, Bangalore (inStem), Bangalore, India.

Christopher Semsarian (C)

Agnes Ginges Centre for Molecular Cardiology at Centenary Institute, The University of Sydney, Sydney, Australia.
Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia.

Robert G Weintraub (RG)

Cardiology Department, Royal Children's Hospital, Melbourne, Australia.
Murdoch Children's Research Institute and Department of Paediatrics, University of Melbourne, Melbourne, Australia.

Richard D Bagnall (RD)

Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia.

Jodie Ingles (J)

Agnes Ginges Centre for Molecular Cardiology at Centenary Institute, The University of Sydney, Sydney, Australia.
Cardio Genomics Program at Centenary Institute, The University of Sydney, Sydney, Australia.

Marta Melé (M)

Life Sciences Department, Barcelona Supercomputing Center, Barcelona, Catalonia, Spain.

Philipp G Maass (PG)

Genetics and Genome Biology Program, The Hospital for Sick Children, Toronto, ON, Canada.
Department of Molecular Genetics, University of Toronto, Toronto, ON, Canada.

James Ellis (J)

Department of Molecular Genetics, University of Toronto, Toronto, ON, Canada.
Developmental and Stem Cell Biology Program, The Hospital for Sick Children, Toronto, ON, Canada.

Stephen W Scherer (SW)

Genetics and Genome Biology Program, The Hospital for Sick Children, Toronto, ON, Canada.
Department of Molecular Genetics, University of Toronto, Toronto, ON, Canada.
The Centre for Applied Genomics, The Hospital for Sick Children, Toronto, ON, Canada.
McLaughlin Centre, University of Toronto, Toronto, ON, Canada.

Seema Mital (S)

Genetics and Genome Biology Program, The Hospital for Sick Children, Toronto, ON, Canada. seema.mital@sickkids.ca.
Ted Rogers Centre for Heart Research, Toronto, ON, Canada. seema.mital@sickkids.ca.
Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada. seema.mital@sickkids.ca.

Classifications MeSH