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2016 ; 29
(3
): 633-58
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Prions: Beyond a Single Protein
#MMPMID27226089
Das AS
; Zou WQ
Clin Microbiol Rev
2016[Jul]; 29
(3
): 633-58
PMID27226089
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Since the term protein was first coined in 1838 and protein was discovered to be
the essential component of fibrin and albumin, all cellular proteins were
presumed to play beneficial roles in plants and mammals. However, in 1967,
Griffith proposed that proteins could be infectious pathogens and postulated
their involvement in scrapie, a universally fatal transmissible spongiform
encephalopathy in goats and sheep. Nevertheless, this novel hypothesis had not
been evidenced until 1982, when Prusiner and coworkers purified infectious
particles from scrapie-infected hamster brains and demonstrated that they
consisted of a specific protein that he called a "prion." Unprecedentedly, the
infectious prion pathogen is actually derived from its endogenous cellular form
in the central nervous system. Unlike other infectious agents, such as bacteria,
viruses, and fungi, prions do not contain genetic materials such as DNA or RNA.
The unique traits and genetic information of prions are believed to be encoded
within the conformational structure and posttranslational modifications of the
proteins. Remarkably, prion-like behavior has been recently observed in other
cellular proteins-not only in pathogenic roles but also serving physiological
functions. The significance of these fascinating developments in prion biology is
far beyond the scope of a single cellular protein and its related disease.
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