Literature DB >> 14563344

The 2373insG mutation in the MYBPC3 gene is a founder mutation, which accounts for nearly one-fourth of the HCM cases in the Netherlands.

Marielle Alders1, Roselie Jongbloed, Wout Deelen, Arthur van den Wijngaard, Pieter Doevendans, Folkert Ten Cate, Vera Regitz-Zagrosek, Hans Peter Vosberg, Irene van Langen, Arthur Wilde, Dennis Dooijes, Marcel Mannens.   

Abstract

AIMS: Hypertrophic cardiomyopathy (HCM) is caused by mutations in genes that encode sarcomeric proteins. In this study we investigated the involvement of the sarcomeric myosin binding protein C in the Dutch HCM population. METHODS AND
RESULTS: We initially screened 22 Dutch index patients for mutations in the MYBPC3 gene, which revealed four different mutations in 14 patients. The 2373insG mutation was identified in 10 apparently unrelated patients. A subsequent screening for the 2373insG mutation in a group of another 237 unrelated HCM patients revealed 50 additional carriers of the same genetic defect. Genotyping with polymorphic repeat markers and intragenic SNPs of the 60 Dutch as well as two German and five North American 2373insG carriers indicated they all share the same haplotype.
CONCLUSION: The 2373insG mutation accounts for almost one-fourth of all HCM cases in the Netherlands (60/259), which is predominantly present in the northwestern part of the country (22/66) and is a founder mutation probably originating from the Netherlands.

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Year:  2003        PMID: 14563344     DOI: 10.1016/s0195-668x(03)00466-4

Source DB:  PubMed          Journal:  Eur Heart J        ISSN: 0195-668X            Impact factor:   29.983


  49 in total

1.  The tale of the three sisters.

Authors:  J Liu; M J Cramer; G E Leenders; J Zhao; P A Doevendans; P G Melman
Journal:  Neth Heart J       Date:  2010-11       Impact factor: 2.380

2.  Founder mutations in hypertrophic cardiomyopathy patients in the Netherlands.

Authors:  I Christiaans; E A Nannenberg; D Dooijes; R J E Jongbloed; M Michels; P G Postema; D Majoor-Krakauer; A van den Wijngaard; M M A M Mannens; J P van Tintelen; I M van Langen; A A M Wilde
Journal:  Neth Heart J       Date:  2010-05       Impact factor: 2.380

3.  Structural abnormalities of the left ventricle in hypertrophic cardiomyopathy mutation carriers detectable before the development of hypertrophy.

Authors:  T Germans; A A M Wilde; C J A van Echteld; O Kamp; Y M Pinto; A C van Rossum
Journal:  Neth Heart J       Date:  2007       Impact factor: 2.380

4.  Recurrent and founder mutations in inherited cardiac diseases in the Netherlands.

Authors:  J P van Tintelen; A A M Wilde; J D H Jongbloed
Journal:  Neth Heart J       Date:  2009-11       Impact factor: 2.380

Review 5.  Hypertrophic cardiomyopathy in daily practice: an introduction on diagnosis, prognosis and treatment.

Authors:  C van der Lee; M J Kofflard; M L Geleijnse; F J Ten Cate
Journal:  Neth Heart J       Date:  2005-12       Impact factor: 2.380

Review 6.  MYBPC3's alternate ending: consequences and therapeutic implications of a highly prevalent 25 bp deletion mutation.

Authors:  Diederik W D Kuster; Sakthivel Sadayappan
Journal:  Pflugers Arch       Date:  2013-12-11       Impact factor: 3.657

Review 7.  Strategies for targeting the cardiac sarcomere: avenues for novel drug discovery.

Authors:  Joshua B Holmes; Chang Yoon Doh; Ranganath Mamidi; Jiayang Li; Julian E Stelzer
Journal:  Expert Opin Drug Discov       Date:  2020-02-18       Impact factor: 6.098

8.  Altered C10 domain in cardiac myosin binding protein-C results in hypertrophic cardiomyopathy.

Authors:  Diederik W D Kuster; Thomas L Lynch; David Y Barefield; Mayandi Sivaguru; Gina Kuffel; Michael J Zilliox; Kyoung Hwan Lee; Roger Craig; Rajasekaran Namakkal-Soorappan; Sakthivel Sadayappan
Journal:  Cardiovasc Res       Date:  2019-12-01       Impact factor: 10.787

9.  Hypertrophic cardiomyopathy family with double-heterozygous mutations; does disease severity suggest doubleheterozygosity?

Authors:  I A W van Rijsingen; J F Hermans-van Ast; Y H J M Arens; S M Schalla; C E M de Die-Smulders; A van den Wijngaard; Y M Pinto
Journal:  Neth Heart J       Date:  2009-12       Impact factor: 2.380

10.  How do hypertrophic cardiomyopathy mutations affect myocardial function in carriers with normal wall thickness? Assessment with cardiovascular magnetic resonance.

Authors:  Tjeerd Germans; Iris K Rüssel; Marco J W Götte; Marieke D Spreeuwenberg; Pieter A Doevendans; Yigal M Pinto; Rob J van der Geest; Jolanda van der Velden; Arthur A M Wilde; Albert C van Rossum
Journal:  J Cardiovasc Magn Reson       Date:  2010-03-15       Impact factor: 5.364

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