[Genetic study of a Parkinson's disease pedigree caused by compound heterozygous mutations in PARK2 gene]

Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2018 Dec 10;35(6):815-818. doi: 10.3760/cma.j.issn.1003-9406.2018.06.009.
[Article in Chinese]

Abstract

Objective: To explore the genetic basis for a Chinese pedigree where three siblings were affected with Parkinson's disease.

Methods: Multiple ligation-dependent probe amplification (MLPA) and next-generation sequencing (NGS) were employed to detect the causative mutation. Sanger sequencing of cDNA was also used for verify the effect of mutation on the transcription of RNA.

Results: Heterozygous deletion of exon 3 of the PARK2 gene was detected by MLPA, while a heterozygous splice site variant c.619-3G>C was detected by NGS. Both mutations were shown to result in aberrant transcripts of the PARK2 gene (loss of exons 3 and 6, respectively) by Sanger sequencing of cDNA. Both mutations have co-segregated with the disease in the pedigree.

Conclusion: Compound heterozygous mutations of the PARK2 gene probably underlie the disease in this pedigree. Identification of the splice site variant c.619-3G>C has expanded the mutation spectrum of the PARK2 gene.

MeSH terms

  • Asian People
  • China
  • DNA Mutational Analysis
  • Exons
  • Heterozygote
  • Humans
  • Mutation
  • Parkinson Disease / genetics*
  • Pedigree
  • Ubiquitin-Protein Ligases / genetics*

Substances

  • Ubiquitin-Protein Ligases
  • parkin protein