[Application of droplet digital PCR technology for genetic testing and prenatal diagnosis of spinal muscular atrophy]

Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2016 Oct;33(5):594-7. doi: 10.3760/cma.j.issn.1003-9406.2016.05.002.
[Article in Chinese]

Abstract

Objective: To explore the clinical application of droplet digital PCR (ddPCR) for genetic testing and prenatal diagnosis of spinal muscular atrophy (SMA) with deletion of SMN1 gene exon 7.

Methods: A total of 138 clinical samples, including 121 peripheral blood, 13 amniotic fluid, 2 umbilical cord blood and 2 chorionic villi from 56 SMA families, were tested by both ddPCR and multiplex ligation-dependent probe amplification (MLPA). Results of the two approaches were analyzed with commercial software QuantaSoft (ddPCR) and Coffalyser (MLPA), respectively.

Results: Among the 138 cases, 25 had two copies, 84 had one copy, and 29 had null copy of exon 7 of the SMN1 gene. The results of ddPCR and MLPA were completely consistent.

Conclusion: As a rapid, precise and economically efficient method, ddPCR will provide a new choice for genetic testing of SMA.

MeSH terms

  • Adult
  • DNA Copy Number Variations
  • Family Health
  • Female
  • Gene Dosage
  • Genetic Predisposition to Disease / genetics*
  • Genetic Testing / methods*
  • Humans
  • Male
  • Multiplex Polymerase Chain Reaction / methods*
  • Muscular Atrophy, Spinal / diagnosis
  • Muscular Atrophy, Spinal / embryology
  • Muscular Atrophy, Spinal / genetics*
  • Pedigree
  • Pregnancy
  • Prenatal Diagnosis / methods*
  • Reproducibility of Results
  • Sensitivity and Specificity
  • Sequence Deletion
  • Survival of Motor Neuron 1 Protein / genetics*

Substances

  • SMN1 protein, human
  • Survival of Motor Neuron 1 Protein