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Bovine spongiform encephalopathy: the prion


Veronica Fowler

Ed van Klink

Synonym(s): BSE, mad cow disease, prion




  • Bovine spongiform encephalopathy (BSE) is an example of a group of diseases called transmissible spongiform encephalopathies (TSEs).
  • TSEs are characterized by a degeneration of brain tissue giving it a sponge-like appearance.


  • The causative agent of BSE is a proteinacious infectious particle prion, a disease-causing form of a normal protein called cellular prion protein (PrPc). 
  • Prions stimulate normal PrPc, to refold into the disease causing form called PrPsc.
  • This leads to a progressive accumulation of the rogue protein within the brain causing the formation of amyloid plaques which leads to neurological and behavioral effects and, eventually to, death.

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Clinical Effects



  • Prion protein (PrPc) is a native protein which is naturally present with the body, although its role is not fully understood. 
  • BSE is caused when an abnormal version of the prion proten (PrPsc) stimulates the naturally occurring PrPc to misfold into the abnormal version.


  • The incubation period of prion diseases is relatively long (5 to 20 years), once symptoms appear the disease progresses rapidly, leading to brain damage and death.


  • There is no evidence to suggest that BSE can be passed between animals under normal circumstances.
  • The BSE crisis of the 1980s was attributed to the consumption, by cattle, of meat and bone meal (MBM) which was contaminated with the infectious prions from cattle affected with BSE, or sheep affected with scrapie. 
  • Maternal transmission may occur, but it is considered as of limited importance.

Pathological effects

  • The pathogenesis is not well defined.
  • Prions have been found in the ileum, CNS and retina.
  • Long incubation period.
  • Hind limb ataxia resulting in poor balance and coordination.
  • Difficulty in rising.
  • Behavioral changes including aggression, anxiety, nervousness or frenzy. Cattle Behavior
  • Decreased milk production and weight loss. Approach to the cow with milk drop


Control via animal

BSE is a notifiable disease within the European Union.
  • Slaughter of infected animals.
    • In some countries the whole herd is slaughtered with the carcasses incinerated.
  • Animal feed that contained animal protein thought to have spread BSE is banned. This is called the feed ban.
  • Specified risk material control measures ensure that the parts of an animal most likely to contain BSE, such as brain and spinal cord, are removed when an animal is slaughtered to ensure they do not enter the food chain.
  • In the UK:
    • All fallen stock or stock that is slaughtered on farm, or in transit, older than 48 months will be tested.
    • All stock older than 72 months that are brought for regular slaughter to an abattoir, where the official veterinarian suspects a health problem, will also be tested.

Control via chemotherapies

  • There is no treatment for BSE.


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Further Reading


Refereed Papers

  • Recent references from PubMed and VetMedResource.
  • Sugiura K, Haga T & Onodera T (2014) Estimating the BSE infection and detectable prevalence in cattle born after 2000 in Japan. Prev Vet Med 115 (3-4), 191-7 PubMed.
  • Westergard L, Christensen H M & Harris D A (2007) The Cellular Prion Protein (PrPC): Its Physiological Function and Role in Disease. Biochimica et biophysica acta 1772 (6), 629-644
  • Buschmann A, Conraths F J, Selhorst T, Schultz J, Kramer M & Groschup M H (2007) Imported and indigenous BSE cases in Germany. Vet Microbiol 123 (4), 287-93 PubMed.
  • Novakofski J, Brewer M S, Mateus-Pinilla N, Killefer J & McCusker R H (2005) Prion biology relevant to bovine spongiform encephalopathy. J Anim Sci 83 (6), 1455-76 PubMed.
  • Heim D & Kihm U (2003) Risk management of transmissible spongiform encephalopathies in Europe. Rev Sci Tech 22 (1), 179-99
  • Doherr M G (2003) Bovine spongiform encephalopathy (BSE)--infectious, contagious, zoonotic or production disease? Acta Vet Scand Suppl 98, 33-42 PubMed.

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