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November 23, 2011Circulation JournalOpen Access

Prevalence and Distribution of Sarcomeric Gene Mutations in Japanese Patients With Familial Hypertrophic Cardiomyopathy

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Population

112 unrelated Japanese proband patients with familial hypertrophic cardiomyopathy (HCM)

Design

Cross-sectional

Key result

Genetic screening of 8 sarcomeric genes in 112 Japanese patients with familial hypertrophic cardiomyopathy identified disease-associated mutations in 43.8% of patients, most frequently in MYBPC3.

Authors

HOHaruna OtsukaTATakuro ArimuraTATadaaki Abe

Discussion

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Overview

May inform genetic testing in Japanese familial HCM; extends sarcomeric mutation prevalence data to Asian cohorts with founder effects noted.

Study Design

Type

Observational (n=112)

Multicenter

Yes

Structured PICO

P
Population
112 unrelated Japanese proband patients with familial hypertrophic cardiomyopathy (HCM)
I
Intervention
Genetic screening of disease-associated mutations in 8 genes for sarcomeric proteins (MYH7, MYBPC3, MYL2, MYL3, TNNT2, TNNI3, TPM1, and ACTC) by direct sequencing
O
Outcome
Frequency and distribution of mutations in 8 sarcomeric genes

In Japanese patients with familial HCM, mutations in sarcomeric genes were found in 43.8% of probands, with MYBPC3 and MYH7 being the most common and showing regional distribution differences.

Limitations

  • Did not analyze the rod region of cardiac beta-myosin heavy chain (exons 26-40 of MYH7), potentially missing some disease-associated mutations.
  • Cannot exclude the possibility that some identified mutations are rare polymorphisms, as in vitro and in vivo functional analyses were not performed.

Cite This Study

Otsuka et al. (2011) conducted an observational in Familial Hypertrophic Cardiomyopathy (n=112). Genetic screening of 8 sarcomeric genes was evaluated on Prevalence of sarcomeric gene mutations. Genetic screening of 8 sarcomeric genes in 112 Japanese patients with familial hypertrophic cardiomyopathy identified disease-associated mutations in 43.8% of patients, most frequently in MYBPC3.

synapsesocial.com/papers/6a081adcef79633196e8a519https://doi.org/10.1253/circj.cj-11-0876
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