Effect of genetic modifiers on cerebral lesions in Fabry disease

Neurology. 2005 Jun 28;64(12):2148-50. doi: 10.1212/01.WNL.0000166000.24321.4F.

Abstract

Fabry disease is associated with increased risk of premature stroke and presumptive ischemic cerebral lesions. In 57 consecutive patients, 35% of whom had lesions on brain MRI, the authors found that genotypes of polymorphisms G-174C of interleukin-6, G894T of endothelial nitric oxide synthase, factor V G1691A mutation, and the A-13G and G79A of protein Z were all significantly associated with cerebral lesions. These findings suggest that these proteins modulate Fabry cerebral vasculopathy.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adolescent
  • Adult
  • Blood Proteins / genetics
  • Brain Ischemia / genetics*
  • Brain Ischemia / metabolism
  • Brain Ischemia / pathology
  • Cerebral Arteries / metabolism*
  • Cerebral Arteries / pathology
  • Cerebral Arteries / physiopathology
  • Cerebral Infarction / genetics*
  • Cerebral Infarction / metabolism
  • Cerebral Infarction / pathology
  • Child
  • DNA Mutational Analysis
  • Encephalitis / genetics
  • Fabry Disease / genetics*
  • Fabry Disease / metabolism
  • Fabry Disease / physiopathology
  • Factor V / genetics
  • Genetic Predisposition to Disease / genetics*
  • Genotype
  • Humans
  • Interleukin-6 / genetics
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Mutation / genetics*
  • Nitric Oxide Synthase Type III / genetics
  • Oxidative Stress / genetics
  • Polymorphism, Genetic / genetics

Substances

  • Blood Proteins
  • Interleukin-6
  • plasma protein Z
  • Factor V
  • Nitric Oxide Synthase Type III