Array-based resequencing assay for mutations causing hypertrophic cardiomyopathy

Clin Chem. 2008 Apr;54(4):682-7. doi: 10.1373/clinchem.2007.099119. Epub 2008 Feb 7.

Abstract

Background: Dissecting the complex genetic basis of hypertrophic cardiomyopathy (HCM) may be key to both better understanding and optimally managing this most prevalent genetic cardiovascular disease. An array-based resequencing (ABR) assay was developed to facilitate genetic testing in HCM.

Methods: An Affymetrix resequencing array and a single long-range PCR protocol were developed to cover the 3 most commonly affected genes in HCM, MYH7 (myosin, heavy chain 7, cardiac muscle, beta), MYBPC3 (myosin binding protein C, cardiac), and TNNT2 [troponin T type 2 (cardiac)].

Results: The assay detected the underlying point mutation in 23 of 24 reference samples and provided pointers toward identifying a G insertion and a 3-bp deletion. The comparability of array-based assay results to conventional capillary sequencing was > or =99.9%. Both techniques detected 1 heterozygous variant that was missed by the other method.

Conclusions: The data provide evidence that ABR can substantially reduce the high workload previously associated with a genetic test for HCM. Therefore, the HCM array could facilitate large-scale studies aimed at broadening the understanding of the genetic and phenotypic diversity of HCM and related cardiomyopathies.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Cardiac Myosins / genetics*
  • Cardiomyopathy, Hypertrophic / genetics*
  • Carrier Proteins / genetics*
  • Heterozygote
  • Humans
  • Myosin Heavy Chains / genetics*
  • Oligonucleotide Array Sequence Analysis
  • Sequence Analysis, DNA
  • Troponin T / genetics*

Substances

  • Carrier Proteins
  • MYH7 protein, human
  • Troponin T
  • myosin-binding protein C
  • Cardiac Myosins
  • Myosin Heavy Chains