Human transmissible spongiform encephalopathies

Ilan Leibovitz, Doron Zamir, Ilia Polychuck, Esther Kahana

Research output: Contribution to journalReview articlepeer-review

1 Scopus citations

Abstract

A new variant of Creutzfeldt-Jakob disease was discovered in 1996. This new variant was found to be related to encephalopathy. There are 5 known human transmissible spongiform encephalopathies, all created by prions. The prion, a pure protein, smaller than a virus is derived from a normal glycoprotein of human cells wall. The function of this protein is unknown but supposed to be connected with copper incorporation into superoxide dismutase. Creutzfeldt-Jakob disease is an early dementive disease with loss of neurons, accumulation of amyloid and spongiform degeneration of the brain. We discuss the epidemiology, diagnosis and therapy of Creutzfeldt-Jakob and its variant. The increased life expectancy will certainly cause these diseases to be more common, thereby having a definite affect on the economy in both developing and developed countries. Future therapy will concentrate on either preventing the creation of pathologic prions or extracting intracellular prions.

Original languageEnglish
Pages (from-to)70-73+76
JournalHarefuah
Volume142
Issue number1
StatePublished - 1 Jan 2003
Externally publishedYes

Keywords

  • Amyloid
  • Creutzfeldt-Jakob disease
  • Prions
  • Spongiform degeneration

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