Granzyme B gene polymorphism associated with subacute sclerosing panencephalitis

Neuropediatrics. 2014 Oct;45(5):309-13. doi: 10.1055/s-0034-1378129. Epub 2014 May 29.

Abstract

Background: Subacute sclerosing panencephalitis (SSPE) is a late complication of measles infection. Immune dysfunction related to genetic susceptibility has been considered in disease pathogenesis. A functional single nucleotide polymorphism (SNP) of granzyme B gene (GZMB) reported in several pathologies may also be involved in susceptibility to SSPE.

Patients and methods: An SNP (rs8192917, G → A, R→Q) was screened in 118 SSPE patients and 221 healthy controls (HC) by polymerase chain reaction-restriction fragment length polymorphism. Frequencies were compared between groups. In vitro production of GZMB was measured in controls with different genotypes.

Results: The SNP had a minor allele (G) frequency of 0.22 in patients and 0.31 in controls. GG genotype was significantly less frequent in patients (odds ratio, 0.23). G allele carriers produced relatively higher levels of GZMB, when stimulated in vitro.

Conclusion: These findings implicate possible effect of this genetic polymorphism in susceptibility to SSPE which needs to be confirmed in bigger populations.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Antigens, CD
  • Child
  • Child, Preschool
  • Cytokines / metabolism
  • Female
  • Genetic Association Studies
  • Genetic Predisposition to Disease / genetics*
  • Genotype
  • Granzymes / genetics*
  • Humans
  • Infant
  • Male
  • Polymorphism, Single Nucleotide / genetics*
  • Subacute Sclerosing Panencephalitis / genetics*
  • Young Adult

Substances

  • Antigens, CD
  • Cytokines
  • Granzymes