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January 1, 2013Circulation JournalOpen Access

Somatic MYH7, MYBPC3, TPM1, TNNT2 and TNNI3 Mutations in Sporadic Hypertrophic Cardiomyopathy

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Why the study?

Do somatic mutations in MYH7, MYBPC3, TPM1, TNNT2, and TNNI3 contribute to the etiology of sporadic hypertrophic cardiomyopathy?

Population

104 unrelated patients with non-familial hypertrophic cardiomyopathy (HCM)

Design

Cross-sectional

Key result

Genetic screening of cardiac tissue and peripheral blood from 104 patients with sporadic hypertrophic cardiomyopathy revealed no evidence of somatic mutations in MYH7, MYBPC3, TPM1, TNNT2, or TNNI3.

Authors

LNLucía NúñezUniversidade da CoruñaJGJuan R. GimenoHeart Failure & TransplantMRMaría Isabel Rodriguez-GarciaHospital La Luz

Discussion

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Overview

Somatic mutations in these sarcomeric genes unlikely in sporadic HCM; challenges prior hypotheses and leaves open alternative etiologies for study.

Study Design

Type

Observational (n=104)

Multicenter

Yes

Structured PICO

Do somatic mutations in MYH7, MYBPC3, TPM1, TNNT2, and TNNI3 contribute to the etiology of sporadic hypertrophic cardiomyopathy?

P
Population
104 unrelated patients with non-familial hypertrophic cardiomyopathy (HCM)
I
Intervention
Mutation screening of 5 genes (MYH7, MYBPC3, TPM1, TNNT2, and TNNI3) in genomic DNA isolated from resected cardiac tissue and corresponding blood
O
Outcome
Presence of somatic mutations in MYH7, MYBPC3, TPM1, TNNT2, and TNNI3surrogate

Main Result

Absolute Event Rate: 0% vs 0%

Somatic mutations in major sarcomeric genes do not appear to be a significant etiologic factor in sporadic hypertrophic cardiomyopathy.

Limitations

  • Lack of functional and familial studies to establish a cause-effect relationship between the novel variants and HCM
  • In silico analysis predictions may be erroneous and must be interpreted carefully

Cite This Study

Núñez et al. (2013) conducted an observational in Hypertrophic cardiomyopathy (n=104). Genetic screening of cardiac tissue vs. Genetic screening of peripheral blood was evaluated on Presence of somatic mutations in MYH7, MYBPC3, TPM1, TNNT2, and TNNI3. Genetic screening of cardiac tissue and peripheral blood from 104 patients with sporadic hypertrophic cardiomyopathy revealed no evidence of somatic mutations in MYH7, MYBPC3, TPM1, TNNT2, or TNNI3.

synapsesocial.com/papers/6a08ee6127ceb0c2a2d61ee6https://doi.org/10.1253/circj.cj-13-0294
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Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Spectrum and clinical manifestations of mutations in genes responsible for hypertrophic cardiomyopathy2012 · 28 citations
  2. 2Mutation spectrum in a large cohort of unrelated consecutive patients with hypertrophic cardiomyopathy2003 · 228 citations
  3. 3Molecular Diagnosis of Hypertrophic Cardiomyopathy (HCM): In the Heart of Cardiac Disease2022 · 35 citations
  4. 4Genetic Dissection of Hypertrophic Cardiomyopathy with Myocardial RNA-Seq2020 · 43 citations
  5. 5Genetics of hypertrophic cardiomyopathy in eastern Finland: few founder mutations with benign or intermediary phenotypes2004 · 60 citations