[Denaturing high-performance liquid chromatography coupled with multiplex PCR for rapid detection of large duplications or deletions in patients with Duchenne muscular dystrophy and spinal muscular atrophy]

Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2012 Dec;29(6):686-9. doi: 10.3760/cma.j.issn.1003-9406.2012.06.013.
[Article in Chinese]

Abstract

Objective: To assess the value of multiplex PCR-denaturing high-performance liquid chromatography (PCR-DHPLC) method for screening large duplications or deletions in patients with Duchenne muscular dystrophy (DMD) and spinal muscular atrophy (SMA).

Methods: DNA was extracted from peripheral venous blood samples from 35 DMD and 6 SMA patients. Large duplications or deletions were screened with multiplex PCR coupled with DHPLC method. The results were validated with testing of positive and negative controls.

Results: Known duplications or deletions in all controls were reliably detected with multiple PCR coupled with DHPLC. Large duplications or deletions were found in 71.4% of 35 DMD patients, which included 5 large duplications and 20 large deletions. For SMA patients, deletions of SMN1 exon 7 were detected in 16 samples.

Conclusion: Multiplex PCR coupled with DHPLC method is an effective and reliable method for detecting large genomic duplications or deletions in patients with DMD or SMA.

Publication types

  • English Abstract

MeSH terms

  • Chromatography, High Pressure Liquid
  • Dystrophin / genetics
  • Gene Deletion*
  • Gene Duplication*
  • Humans
  • Multiplex Polymerase Chain Reaction
  • Muscular Atrophy, Spinal / diagnosis*
  • Muscular Atrophy, Spinal / genetics*
  • Muscular Dystrophy, Duchenne / diagnosis*
  • Muscular Dystrophy, Duchenne / genetics*
  • Survival of Motor Neuron 1 Protein / genetics

Substances

  • Dystrophin
  • Survival of Motor Neuron 1 Protein