Functional MxA promoter polymorphism associated with subacute sclerosing panencephalitis

Neurology. 2004 Feb 10;62(3):457-60. doi: 10.1212/01.wnl.0000106940.95749.8e.

Abstract

Background: The antivirally active MxA protein is induced by interferon (IFN) alpha/beta and inhibits the replication of single-stranded RNA viruses including measles virus (MV). The authors investigated whether the MxA gene contributed to the development of subacute sclerosing panencephalitis (SSPE) in Japanese individuals.

Methods: Single-nucleotide polymorphisms (SNP) in the promoter region of the MxA gene were screened, association studies were performed between two SNP and SSPE, and then a functional difference in the promoter activities of the two SNP was investigated by a dual luciferase reporter assay.

Results: Four SNP were found (-88 G/T, -123 C/A, -200 T/C, and -213 G/T), and SSPE patients exhibited a higher frequency of both the -88T allele and the -88TT genotype than controls (p = 0.040 and 0.003). The IFN-induced up-regulation of the MxA promoter activity of the sequence with -88T was found to be significantly higher than that with G.

Conclusions: MxA promoter -88 G/T SNP may confer host genetic susceptibility to SSPE in Japanese individuals. The finding that homozygotes of the MxA -88T allele with a high MxA-producing capability were more frequently seen in SSPE patients suggests that the MxA protein promotes the establishment of persistent MV infection of neural cells.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Alleles
  • Asian People / genetics
  • Base Sequence
  • Child
  • Child, Preschool
  • Female
  • GTP-Binding Proteins / genetics
  • GTP-Binding Proteins / physiology*
  • Gene Expression Regulation / drug effects
  • Genes, Reporter
  • Genetic Predisposition to Disease
  • Haplotypes
  • Humans
  • Interferon-alpha / pharmacology
  • Japan
  • Linkage Disequilibrium
  • Male
  • Measles virus / physiology
  • Molecular Sequence Data
  • Myxovirus Resistance Proteins
  • Neurons / virology
  • Polymorphism, Single Nucleotide*
  • Promoter Regions, Genetic / drug effects
  • Promoter Regions, Genetic / genetics*
  • Subacute Sclerosing Panencephalitis / genetics*
  • Subacute Sclerosing Panencephalitis / virology
  • Virus Latency / genetics
  • Virus Latency / physiology

Substances

  • Interferon-alpha
  • MX1 protein, human
  • Myxovirus Resistance Proteins
  • GTP-Binding Proteins