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D. Carleton Gajdusek, a virologist who won the 1976 Nobel Prize in medicine for his work on the mysterious epidemics now known as prion diseases, died last week in Tromso, Norway.
Silicon smarts ReprintsThe protein from which prions are formed (known, with startling originality, as prion protein) is found in almost all tissues in man and beast.
Prion diseases are associated with the accumulation of an abnormal glycoprotein known as prion protein (PrP) in the brain.
It is for thus reason that long stretches of glutamine and asparagine repeats have been identified as prion domains.
Different prion-isolates show specific clinical and biochemical traits which are referred to as prion strains [9].
Long SAARs can be tolerated in bacteria and yeast, and it might be that the classification of these low complexity regions as prion domains is misleading.
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Repeats of these amino acids are associated with neurodegenerative diseases in humans and for this reason they are often annotated as prion-like domains [22].
This finding can be interpreted as prion-like transmission or as seeding process.
We showed that BSE infection of primates may occur as prion-amyloid cardiomyopathy.
Strains that were respiration competent were stored as prion-negative strains in glycerol stocks; strains that were not respiration-competent were excluded from further experiments.
We report on the novel clinicopathologic characteristics of vCJD as prion-amyloid cardiomyopathy in 1 of 3 macaques inoculated with BSE.
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