[Human transmissible spongiform encephalopathies]

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Updated: Sep 26, 2026

Assessing Transmissible Spongiform Encephalopathy Species Barriers with an In Vitro Prion Protein Conversion Assay
11:41

Assessing Transmissible Spongiform Encephalopathy Species Barriers with an In Vitro Prion Protein Conversion Assay

Published on: March 10, 2015

Ilan Leibovitz1, Doron Zamir, Ilia Polychuck

  • 1Department of Medicine, Barzilai Medical Center, Ashkelon, Ben Gurion University of the Negev.

Harefuah
|March 22, 2003
PubMed

Insights

A new variant of Creutzfeldt-Jakob disease (vCJD) linked to encephalopathy emerged in 1996. This prion disease, affecting the brain, is becoming more common due to increased life expectancy, impacting global economies.

Area of Science:

  • Neurology
  • Prion Science
  • Infectious Diseases

Context:

  • Creutzfeldt-Jakob disease (CJD) is a rare, fatal neurodegenerative disorder.
  • A novel variant, variant Creutzfeldt-Jakob disease (vCJD), emerged in 1996, linked to bovine spongiform encephalopathy.
  • Prions, misfolded proteins, are the causative agents of transmissible spongiform encephalopathies (TSEs).

Purpose:

  • To review the epidemiology, diagnosis, and therapy of CJD and vCJD.
  • To discuss the nature of prions and their role in human TSEs.
  • To explore the potential economic impact and future therapeutic strategies for prion diseases.

Summary:

  • vCJD, a new variant of Creutzfeldt-Jakob disease, was identified in 1996 and is associated with encephalopathy.
  • Prions, infectious proteins smaller than viruses, cause the 5 known human transmissible spongiform encephalopathies.
  • CJD involves neuronal loss, amyloid accumulation, and brain degeneration; its increasing prevalence due to longer lifespans necessitates economic consideration.

Impact:

  • Rising incidence of CJD and vCJD due to increased life expectancy will impact global economies.
  • Future therapies aim to prevent pathological prion formation or facilitate intracellular prion clearance.
  • Understanding prion diseases is crucial for public health and economic planning.

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