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Prions. Molecular and Cellular Biology
International Journal of Food Microbiology 61 (2000) 211 www.elsevier.nl / locate / ijfoodmicro
Book review Prions. Molecular and Cellular Biology D. A. Harris. Horizon Scientific Press, England, 1999; 218 pages, hard back; £74.99 or $129.99; ISBN 1 -898 -486 -07 -7; http: / /www.horizonpress.com ]]]]]]]] A team of 50 international experts are cutting the edge of research in their field. The work described by these authors lays the groundwork for resolution of some of the most pressing questions that remains in our understanding of prion phenomena from microorganisms to mammals. Focus is on the cellular, biochemical, and genetic aspects. BSE, Bovine Spongiform Encephalopathy, in cattle is one of the most important economic problems that has occurred in Europe during the last 20 years. It is in the same token the most disastrous example on how a change in technology in the food chain may have impact on the society with regard to human and animal health. Meat and Bone Meal, MBM, derived from sheep suffering from scrabie, has been identified as the causative agent of BSE. Before the 1980s bovine and ovine offal was converted into tallow and MBM using organic solvent treatment and heat. A crises in the tallow business and other factors, such as an increased sheep population and increased fuel prices, led to abandoning of the organic solvent extraction resulting in a significantly lower heating of MBM. TSA, Transmissible Spongiform Encephalopathy Agents, Prions, are very resistant to heat, but they are susceptible to lipid solvents, leading to incomplete inactivation. The book extensively deals with the pertinent question: Is the new variant of Creutzfeld-Jacob disease (vCJD) due to contamination of humans with the BSE agent? This is enlightened upon throughout the book on a scientific high level with
very balanced viewpoints. The above question is thus by one of the authors answered as follows: ‘‘Despite strong probability of infection of humans with the BSE agent, the link between BSE agent and vCJD is not 100% proven, but in terms of public health, one must consider that BSE is transmissible to humans’’. In transmissible spongiform encephalopathies (TSE), the prion diseases, the endogenous proteasesensitive, prion protein (PrP-sen) of the host is converted to an abnormal pathogenic form that has a characteristic partial protease resistance (PrP-res). How the cellular prion protein (PrP C ) is converted into its pathogenic form PrP Sc form, is the subject for several chapters, mostly based on molecular approaches. Inherited prion diseases is naturally explained and it is said that approximately 10–15% of all human prion diseases are familial and the result from mutations. A chapter on The Human Genetic Prion Diseases is devoted to this interesting subject, analysing a number of human prion diseases explaining the Pathobiology of familial prion diseases. Other interesting questions as Treatment perspectives is explained and in fact some possibilities seem to exist, as the use of iododoxorubicin (IDX) is maintained to be able to reduce amyloid deposit. The book is essential reading for all researchers working in the field. The book however also appeals to all persons interested in knowing the state of art of prion diseases explained by scientists, devoid of all the emotional aspect which has governed the discussion in many years. Truth and uncertainties are put forward in the book balanced and fair.
0168-1605 / 00 / $ – see front matter 2000 Elsevier Science B.V. All rights reserved. PII: S0168-1605( 00 )00391-3
Niels Skovgaard Jakob Knudsensvej 18 3460 Birkerød Denmark
Book review Prions. Molecular and Cellular Biology D. A. Harris. Horizon Scientific Press, England, 1999; 218 pages, hard back; £74.99 or $129.99; ISBN 1 -898 -486 -07 -7; http: / /www.horizonpress.com ]]]]]]]] A team of 50 international experts are cutting the edge of research in their field. The work described by these authors lays the groundwork for resolution of some of the most pressing questions that remains in our understanding of prion phenomena from microorganisms to mammals. Focus is on the cellular, biochemical, and genetic aspects. BSE, Bovine Spongiform Encephalopathy, in cattle is one of the most important economic problems that has occurred in Europe during the last 20 years. It is in the same token the most disastrous example on how a change in technology in the food chain may have impact on the society with regard to human and animal health. Meat and Bone Meal, MBM, derived from sheep suffering from scrabie, has been identified as the causative agent of BSE. Before the 1980s bovine and ovine offal was converted into tallow and MBM using organic solvent treatment and heat. A crises in the tallow business and other factors, such as an increased sheep population and increased fuel prices, led to abandoning of the organic solvent extraction resulting in a significantly lower heating of MBM. TSA, Transmissible Spongiform Encephalopathy Agents, Prions, are very resistant to heat, but they are susceptible to lipid solvents, leading to incomplete inactivation. The book extensively deals with the pertinent question: Is the new variant of Creutzfeld-Jacob disease (vCJD) due to contamination of humans with the BSE agent? This is enlightened upon throughout the book on a scientific high level with
very balanced viewpoints. The above question is thus by one of the authors answered as follows: ‘‘Despite strong probability of infection of humans with the BSE agent, the link between BSE agent and vCJD is not 100% proven, but in terms of public health, one must consider that BSE is transmissible to humans’’. In transmissible spongiform encephalopathies (TSE), the prion diseases, the endogenous proteasesensitive, prion protein (PrP-sen) of the host is converted to an abnormal pathogenic form that has a characteristic partial protease resistance (PrP-res). How the cellular prion protein (PrP C ) is converted into its pathogenic form PrP Sc form, is the subject for several chapters, mostly based on molecular approaches. Inherited prion diseases is naturally explained and it is said that approximately 10–15% of all human prion diseases are familial and the result from mutations. A chapter on The Human Genetic Prion Diseases is devoted to this interesting subject, analysing a number of human prion diseases explaining the Pathobiology of familial prion diseases. Other interesting questions as Treatment perspectives is explained and in fact some possibilities seem to exist, as the use of iododoxorubicin (IDX) is maintained to be able to reduce amyloid deposit. The book is essential reading for all researchers working in the field. The book however also appeals to all persons interested in knowing the state of art of prion diseases explained by scientists, devoid of all the emotional aspect which has governed the discussion in many years. Truth and uncertainties are put forward in the book balanced and fair.
0168-1605 / 00 / $ – see front matter 2000 Elsevier Science B.V. All rights reserved. PII: S0168-1605( 00 )00391-3
Niels Skovgaard Jakob Knudsensvej 18 3460 Birkerød Denmark