Document Detail


A rare variant in MYH6 is associated with high risk of sick sinus syndrome.
MedLine Citation:
PMID:  21378987     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Through complementary application of SNP genotyping, whole-genome sequencing and imputation in 38,384 Icelanders, we have discovered a previously unidentified sick sinus syndrome susceptibility gene, MYH6, encoding the alpha heavy chain subunit of cardiac myosin. A missense variant in this gene, c.2161C>T, results in the conceptual amino acid substitution p.Arg721Trp, has an allelic frequency of 0.38% in Icelanders and associates with sick sinus syndrome with an odds ratio = 12.53 and P = 1.5 × 10⁻²⁹. We show that the lifetime risk of being diagnosed with sick sinus syndrome is around 6% for non-carriers of c.2161C>T but is approximately 50% for carriers of the c.2161C>T variant.
Authors:
Hilma Holm; Daniel F Gudbjartsson; Patrick Sulem; Gisli Masson; Hafdis Th Helgadottir; Carlo Zanon; Olafur Th Magnusson; Agnar Helgason; Jona Saemundsdottir; Arnaldur Gylfason; Hrafnhildur Stefansdottir; Solveig Gretarsdottir; Stefan E Matthiasson; Gu Mundur Thorgeirsson; Aslaug Jonasdottir; Asgeir Sigurdsson; Hreinn Stefansson; Thomas Werge; Thorunn Rafnar; Lambertus A Kiemeney; Babar Parvez; Raafia Muhammad; Dan M Roden; Dawood Darbar; Gudmar Thorleifsson; G Bragi Walters; Augustine Kong; Unnur Thorsteinsdottir; David O Arnar; Kari Stefansson
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Validation Studies     Date:  2011-03-06
Journal Detail:
Title:  Nature genetics     Volume:  43     ISSN:  1546-1718     ISO Abbreviation:  Nat. Genet.     Publication Date:  2011 Apr 
Date Detail:
Created Date:  2011-03-29     Completed Date:  2011-05-09     Revised Date:  2011-09-26    
Medline Journal Info:
Nlm Unique ID:  9216904     Medline TA:  Nat Genet     Country:  United States    
Other Details:
Languages:  eng     Pagination:  316-20     Citation Subset:  IM    
Affiliation:
deCODE Genetics, Sturlugata 8, Reykjavik, Iceland. hilma.holm@decode.is
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MeSH Terms
Descriptor/Qualifier:
Adult
Aged
Aged, 80 and over
Cardiac Myosins / genetics*
Case-Control Studies
Female
Genetic Predisposition to Disease
Genetic Variation
Genome-Wide Association Study
Heart Diseases / genetics
Heart Rate / genetics
Heterozygote
Humans
Iceland
Male
Middle Aged
Mutation, Missense*
Myosin Heavy Chains / genetics*
Odds Ratio
Oligonucleotide Array Sequence Analysis
Penetrance
Polymorphism, Single Nucleotide
Sick Sinus Syndrome / genetics*,  physiopathology
Grant Support
ID/Acronym/Agency:
HL092217/HL/NHLBI NIH HHS; R01 HL092217-03/HL/NHLBI NIH HHS; U19 HL065962-10/HL/NHLBI NIH HHS; U19HL065962/HL/NHLBI NIH HHS
Chemical
Reg. No./Substance:
0/MYH6 protein, human; 0/Myosin Heavy Chains; EC 3.6.1.-/Cardiac Myosins
Comments/Corrections
Comment In:
Nat Genet. 2011 Apr;43(4):287-8   [PMID:  21445070 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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