Journal of hospital Pula, Vol. 1 No. 1, 2004.
Review article
PRIONS AND PRION DISEASES
Dubravko Marković
Matija Marković
Abstract
A few typical disorders mostly manifesting by gate disturbances, muscle wasting and progressive immobility to the death, have been known in animals all around the world (sheep, goats, cats, deer’s, minks) for centuries. Cowboys in USA sometimes used the term “loco” for the cow with gait disturbances and so called madness. This word was also used for the “mad people”. In the 20-ties of the last century two German doctors described the progressive dementia different from others dementia’s like the Paretic dementia or Alzheimer disease. The disorders later known as Creutzfeld-Jakob (CJD) disease. Similar disease was known in New Guinea for centuries as kuru. Disease have been found only among the Fore tribe in Papua. In the 60-ties the disease was transmitted to the chimpanzee. The term transmissible spongiform encephalopathy (TSE) has been used. In the recent years after the bovine spongiform encephalopathy (BSE) was followed by increased number of patients with CJD started the panic, mostly caused by journalists looking for sensations. Scientific studies and debates lasted for months and years. The prion theory is based on the finding of an infection-related, low molecular weight protein in TSE tissues. Several lines of evidence suggest that a glycosylated protease-resistant protein (PrP) is a component of the infectious particle.” Fractions with high infection titres contain one protein, PrP 27-30, as determined by polyacrylamide gel electrophoresis. The name prion comes from the short for “proteinaceous infectious particle” Prions are thought to transform normal proteins into infectious agents, killing ones by altering the healthy to sick molecules. In second part of the article the animal and humanprion diseases are shortly described.
Keywords
Hrčak ID:
7607
URI
Publication date:
15.12.2004.
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