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Book Review
Prions and Prion Diseases:
Current Perspectives
Glenn C. Telling, Editor
Horizon Bioscience, Norfolk, England
ISBN: 0-9545232-6-1
Pages: 307, Price: U.S. $180, GB £95
Suggested
citation for this article
Prion diseases, also known as transmissible spongiform encephalopathies,
are rapidly progressive, uniformly fatal brain diseases that can infect
humans and animals, including cattle, sheep, goats, mink, deer, elk, cats,
and zoo ungulates. In humans, prion diseases can occur as a sporadic or
inherited disease, or as a result of iatrogenic transmission. Prion diseases
generated great public concern after an outbreak of bovine spongiform
encephalopathy occurred in many European countries and scientific evidence
indicated its transmission to humans.
Research in prion diseases is hampered by certain unconventional properties
of the presumed etiologic agent and the long incubation period associated
with these diseases. Most conventional laboratory methods used to study
viruses and bacteria may not be applicable. In the past, the etiologic
agent of transmissible spongiform encephalopathies was believed to be
a slow virus, primarily because of its transmissibility, ability to retain
infectivity after filtration, and long incubation period. The successful
transmission of scrapie, a centuries-old prion disease of sheep, to mice
in 1961 greatly facilitated identification and characterization of the
scrapie agent. Several characteristics of the scrapie agent suggest that
the agent is not a virus but is likely composed primarily of a protein.
The agent's characteristics include the absence of disease-specific nucleic
acids; resistance to radiation, nucleases, and standard sterilization
and disinfection methods; and inactivation by protein-modifying procedures.
These observations and purification of the scrapie prion in the early
1980s led to widespread acceptance of the prion hypothesis.
Since the 1980s, both the scope and nature of prion disease research
has progressed rapidly. The economic and human cost associated with the
bovine spongiform encephalopathy outbreak fueled the need to better understand
the etiologic agent of prion diseases and their basic transmission mechanism.
Prions and Prion Diseases: Current Perspectives summarizes the advances
in prion disease research. It expands on a previous volume edited by David
Harris that was published in 1999 under the title Prions: Molecular and
Cellular Biology. The book's 10 chapters describe the biochemical and
molecular features of prions and the normal prion protein, various laboratory
methods for studying prions, and advances in the pathogenesis and immunology
of prion diseases.
Chapters 2 through 6 detail laboratory methods developed to study the
unconventional agent of prion diseases. Chapter 2 describes a cell-free
conversion reaction system to study how pathogenic prions associated with
different species interact with host cellular prion protein. Such systems
have been used to study the biochemical mechanisms of prion diseases and
can potentially be used to screen new therapies for their effectiveness
against prion diseases. Chapter 3 describes the mechanisms underlying
the biosynthesis and cell biology of the cellular prion protein by using
cell culture systems. Understanding the detailed biochemical properties
of the cellular prion protein will help show the molecular basis of its
interaction with, and conversion to, the pathogenic prions. Subsequent
chapters in the book describe other laboratory methods, including transgenic
mouse models, which can be used to investigate the transmissibility of
prions among different species, the extent and degree of the "species
barrier," the mechanism of prion propagation, and prion disease pathogenesis.
Overall, the book provides a wealth of information on the progress made
in understanding the molecular, immunologic, and genetic aspects of prion
diseases and the laboratory methods used to study them. This book will
be valuable to prion disease researchers, to scientists who want to gain
more knowledge about the progress made in understanding the mechanisms
of prion propagation, and to persons just beginning to study these unconventional,
fatal brain diseases.
Ermias D. Belay*
*Centers for Disease Control and Prevention, Atlanta, Georgia, USA
Suggested citation
for this article:
Belay ED. Prions and
prion diseases: current perspectives [book review]. Emerg Infect Dis [serial
on the Internet] 2004 Dec [date cited]. Available from http://www.cdc.gov/ncidod/EID/vol10no12/04-0847.htm
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