BSE and vCJD cause disturbance to uric acid levels

Tamuna Lekishvili, Judyth Sassoon, Andrew R Thompsett, Alison Green, James W Ironside, David R Brown

Research output: Contribution to journalArticlepeer-review


Bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) are two new members of the family of neurodegenerative conditions termed prion diseases. Oxidative damage has been shown to occur in prion diseases and is potentially responsible for the rapid neurodegeneration that is central to the pathogenesis of these diseases. An important nonenzymatic antioxidant in the brain is uric acid. Analysis of uric acid in the brain and cerebrospinal fluid (CSF) of cases of BSE and CJD showed a specific reduction in CSF levels for both BSE and variant CJD, but not sporadic CJD. Further studies based on cell culture experiments suggested that uric acid in the brain was produced by microglia. Uric acid was also shown to inhibit neurotoxicity of a prion protein peptide, production of the abnormal prion protein isoform (PrP(Sc)) by infected cells, and polymerization of recombinant prion protein. These findings suggest that changes in uric acid may aid differential diagnosis of vCJD. Uric acid could be used to inhibit cell death or PrP(Sc) formation in prion disease.

Original languageEnglish
Pages (from-to)233-44
Number of pages12
JournalExperimental neurology
Issue number1
Publication statusPublished - Nov 2004


  • Animals
  • Brain
  • Brain Chemistry
  • Cattle
  • Cell Survival
  • Cells, Cultured
  • Creutzfeldt-Jakob Syndrome
  • Dose-Response Relationship, Drug
  • Encephalopathy, Bovine Spongiform
  • Humans
  • Microglia
  • Neurons
  • Peptide Fragments
  • PrPSc Proteins
  • Prions
  • Uric Acid


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