Broadening spectrum of bovine spongiform encephalopathies

M. Füzi, P. Horváth

Research output: Contribution to journalReview article

Abstract

Until recently the etiology of bovine spongiform encephalopathy (BSE) was considered uniform. The infectious agent was thought to be a single strain of prion (posttranslationally altered form of normal prion protein: PrPSc) retaining its biochemical and biological characteristics during interspecies transmission. However, alternate PrPSc signatures through large-scale screening have recently been detected. In addition, genetic alterations governing susceptibility to prion infection and a mutation (E211K) capable of eliciting spontaneous BSE have been demonstrated. Thus, the spectrum of BSEs have broadened and three PrPSc variants (BSE-C, BSE-H and BSE-L) are now defined. Moreover, a new condition resembling BSE, idiopathic brainstem neuronal chromatolysis (IBNC), has been described that may also turn out to be a prion disease. Since one of the new BSE variants, L-type BSE, proved highly pathogenic detection and further characterization of the new conditions are essential.

Original languageEnglish
Pages (from-to)53-60
Number of pages8
JournalActa microbiologica et immunologica Hungarica
Volume56
Issue number1
DOIs
Publication statusPublished - Mar 1 2009

Keywords

  • BSE strain variants (BSE-C, BSE-H and BSE-L)
  • Bovine spongiform encephalopathy (BSE)
  • Idiopathic brainstem neuronal chromatolysis (IBNC)
  • Prion protein (PrP)

ASJC Scopus subject areas

  • Immunology and Microbiology(all)

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